Download A Guide For Patients, Survivors, and Loved Ones

Understanding
Hodgkin Lymphoma
A Guide For Patients, Survivors, and Loved Ones
Fourth Edition
This guide is an educational resource compiled by the Lymphoma
Research Foundation (LRF) that provides general information on adult
Hodgkin lymphoma. Publication of this information is not intended
to take the place of medical care or the advice of a patient’s doctor.
Patients are strongly encouraged to talk to their doctors for complete
information on how their disease should be diagnosed, treated,
and followed. Before starting treatment, patients should discuss the
potential benefits and side effects of cancer therapy.
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115 Broadway, Suite 1301
New York, NY 10006
(212) 349-2910 phone
(212) 349-2886 fax
Helpline:
(800) 500-9976; [email protected]
Website:
www.lymphoma.org
Email:
[email protected]
This patient guide is supported through unrestricted educational grants from:
© 2012 Lymphoma Research Foundation.
Information contained herein is the property of the Lymphoma Research Foundation (LRF).
Any portion may be reprinted or reproduced provided that LRF is acknowledged to be the source.
ACKNOWLEDGMENTS
The Lymphoma Research Foundation wishes to acknowledge those
individuals listed below who have given generously of their time and
expertise. We thank them for their contributions, editorial wisdom,
and advice, which have truly enhanced this publication. The review
committee guided the content and development of this publication.
Without their dedication and efforts, this publication would not have
been possible. We hope those in the lymphoma community will now
be better informed and have a better understanding of their illness
because of the gracious efforts of those involved in the planning and
execution of this comprehensive disease guide.
Review Committee
Ranjana Advani, MD, Stanford University Medical Center
Nancy L. Bartlett, MD, Washington University School of Medicine
Sven de Vos, MD, PhD, UCLA’s Jonsson Comprehensive Cancer
Center
Randy Gascoyne, MD, FRCPC, British Columbia Cancer Agency
David J. Straus, MD, Memorial Sloan-Kettering Cancer Center
Anas Younes, MD, The University of Texas, MD Anderson Cancer
Center
ii
Understanding Hodgkin Lymphoma
TA B L E O F C O N T E N T S
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
Part 1 — Learning the Basics. . . . . . . . . . . . . . . . . . . . . . . . . 2
Chapter 1: Understanding Hodgkin Lymphoma . . . . . . . . . . . . 2
Chapter 2: Seeking Medical Help . . . . . . . . . . . . . . . . . . . . . . . 12
Chapter 3: Getting a Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . 15
Chapter 4: Work-up Before Treatment Can Begin . . . . . . . . . 21
Part 2 — Treatment of Hodgkin Lymphoma . . . . . . . . . . 27
Chapter 5: What You Should Know
Before Starting Treatment . . . . . . . . . . . . . . . . . . . 27
Chapter 6: Treatments for Hodgkin Lymphoma . . . . . . . . . . . 40
Part 3 — Side Effects and Living With Cancer . . . . . . . 59
Chapter 7: Common Treatment Side Effects . . . . . . . . . . . . . 59
Chapter 8: Managing Your Life During
and After Treatment . . . . . . . . . . . . . . . . . . . . . . . . 77
Part 4 — Clinical Trials and Advances in Treatment . . 82
Chapter 9: Overview of Clinical Trials . . . . . . . . . . . . . . . . . . . 82
Chapter 10: Advances in Treatment of Patients
With Hodgkin Lymphoma . . . . . . . . . . . . . . . . . . 87
About the Lymphoma Research Foundation . . . . . . . . . . . . . . . . . . . 89
Understanding Hodgkin Lymphoma
iii
INTRODUCTION
The purpose of this booklet is to help patients with Hodgkin lymphoma
become active participants in their healthcare decisions. Chapters in
this book address different issues faced by these patients, including:
what to expect during diagnosis, work-up, and treatment; how to cope
with treatment side effects; and what questions to ask doctors.
1
Understanding Hodgkin Lymphoma
Part 1 — Learning the Basics
Chapter 1: Understanding Hodgkin Lymphoma
The sections in this chapter will explain these and other terms that will
help you understand HL and how it affects a person’s health. A better
understanding of the disease will help patients take a more active role
in deciding the course of their treatment.
What is cancer?
Cancer is a group of diseases that develop when abnormal cells gain
the ability to divide abnormally and to spread to areas in the body
where they are not normally found.
Your body is made up of many different types of specialized cells that
are organized in tissues and organs to perform all the different tasks
needed to stay healthy. To keep things running smoothly, the cells in
your body grow, work, and divide in a very controlled fashion.
All of these cells also have a limited lifespan. Normally, a self-destruct
mechanism is triggered when a cell becomes too old or when it
stops working properly. However, sometimes damage to the genetic
material (DNA) of a cell gives it the ability to override this self-destruct
mechanism, allowing these abnormal cells to live longer than normal.
Unless the body’s immune system gets rid of them, these abnormal
cells can multiply and cause cancer.
Learning the Basics
2
Part 1
Hodgkin lymphoma (HL) is a type of cancer that affects specialized
white blood cells called lymphocytes. Lymphocytes work together
with other cells in the immune system to defend the body against
invasion by bacteria, viruses, parasites, and other foreign substances.
Lymphocytes travel around the body in blood vessels and in a
separate network of vessels called the lymphatic system.
HOW CANCER FORMS INSIDE THE BODY
Abnormal
Cells Evade the
Immune System
Abnormal
Cells Multiply
(Cancer)
Tumors May Form
(Groups of
Abnormal Cells)
Most cancers are named after the organ or cell type of origin. For
example, a cancer that started in the pancreas is called pancreatic
cancer and a cancer that started in lymphocytes is called a lymphoma.
3
Understanding Hodgkin Lymphoma
Why is there no single cure for cancer?
What is the lymphatic system?
As shown in the picture on page 5, the lymphatic system is a
circulatory system that is made up of a spidery network of thin tubes
called lymph vessels. Similar to blood vessels, lymph vessels branch
out into all tissues of the body. While people can clearly see blood
vessels, especially at their wrists and on the top of their hand, the
lymph vessels are invisible to the naked eye.
Learning the Basics
4
Part 1
Cancer refers to a large group of very complicated diseases. A tricky
aspect of cancer is that there are many different ways for a cell to
become abnormal enough to develop into a cancer cell. Because of
this, the path taken by a liver cell to become a cancerous liver cell is
quite different from that taken by a lymphocyte to become HL. This
is why a treatment that works against one type of cancer may not
necessarily work against another. There are also smaller, but still
important, differences in the cancer cells found in different patients
diagnosed with the same type of cancer. Because of this, a treatment
that may work very well in one patient may not have the same positive
effect in another. These are some of the reasons why most cancers
are much harder to treat or prevent than, for example, bacterial
or fungal infections, which typically are caused by a single type of
organism.
ANATOMY OF THE IMMUNE SYSTEM
The immune system is the body’s defense against disease.
Tonsil
Thymus
Diaphragm
Lymph
nodes
Spleen
Lymph
vessels
5
Understanding Hodgkin Lymphoma
Lymph fluid flows through lymph nodes and specialized lymph tissues
such as the spleen, tonsils, bone marrow, and thymus gland. Lymph
nodes filter lymph fluid, removing bacteria, viruses, and other foreign
substances from the body. If a large number of foreign substances
are filtered through a node or series of nodes, swelling may occur and
the nodes may become tender to the touch. Most swollen nodes are a
reaction to infection and are not cancerous.
What is a lymphoma?
A lymphoma is a cancer that affects lymphocytes, a type of white blood
cell. Lymphocytes travel through the blood and lymphatic system to
defend the body against foreign invaders like bacteria and viruses.
There are 2 major categories of lymphomas: Hodgkin lymphoma
(HL) and non-Hodgkin lymphoma (NHL). NHL is approximately 8
times more common than HL; according to the American Cancer
Society, each year in the United States approximately 9,000 adults
are diagnosed with HL and over 70,000 with NHL. Both of these major
categories of lymphoma are further subdivided into several types that
are different in the way they develop and spread, and in how affected
patients are treated.
Lymphomas usually develop when a change, or mutation, occurs
within a lymphocyte, causing the abnormal cell to replicate faster than,
or live longer than, a normal lymphocyte. Like normal lymphocytes,
cancerous lymphocytes can travel through the blood and lymphatic
system and spread and grow in many parts of the body, including the
lymph nodes, spleen, bone marrow, and other organs. In advanced
stages, most types of HL are found throughout the body by the time a
patient is diagnosed with the disease.
Learning the Basics
6
Part 1
Lymph vessels carry lymph, a type of liquid that contains lymphocytes.
Within this huge network of vessels are groups of small, bean-shaped
organs called lymph nodes. Thousands of lymph nodes are found
throughout the body, including the elbows, neck, armpits, chest,
abdomen, and groin.
What is Hodgkin lymphoma?
HL (previously called Hodgkin’s disease) is a type of cancer that starts
in lymphocytes. HL is named after Dr. Thomas Hodgkin, a British
physician who first described the disease in 1832. Both children and
adults can develop HL; the disease is most common in younger adults
aged 15-35 and older adults aged 55 and older.
As a result of advances in the diagnosis and treatment of the disease,
over 80% of patients with HL are cured.
How does Hodgkin lymphoma develop?
HL develops when a lymphocyte (usually a type of lymphocyte called
a B cell) becomes abnormal (cancerous). These abnormal cells are
called Reed-Sternberg (R-S) cells, named after the 2 scientists,
Dorothy Reed and Carl Sternberg, who provided the first definitive
microscopic descriptions of HL. Most people with HL have R-S cells,
although other abnormal cell types may also be present. R-S cells are
the defining cells of HL, although they constitute only a small fraction
of the overall tumor mass, and they are not dividing cells. R-S cells
produce factors that attract many inflammatory cells that make up
most of the overall tumor burden. Due to this circumstance, HL initially
was not recognized as a cancer but thought of as an infection.
7
Understanding Hodgkin Lymphoma
A REED-STERNBERG CELL
Part 1
Reed-Sternberg
cell
Normal lymphocyte
Having R-S cells alone does not necessarily mean that a person has
HL. To confirm a diagnosis, the lymphatic tissue sample must also
contain other cells and features that are characteristic of HL. To help
make the diagnosis, a lymphoma pathologist (a doctor specialized
in recognizing lymphoid cancers by examining tissues and organs
under a microscope) will use tests that look for cell surface markers
(antigens) that identify specific cell types of HL, and may also use
more sophisticated molecular tests to help secure a confident
diagnosis.
HL usually starts in the lymph nodes and may be first noticed in the
neck, above or below the collarbone, under the arms, or in the chest.
Because lymph tissues all over the body are connected, abnormal
(cancerous) lymphocytes can circulate in the lymphatic vessels,
causing the lymphoma to spread from one lymph node to another
throughout the body. HL can also spread to other areas and organs
Learning the Basics
8
outside the lymph system. Unlike the more common NHL, HL tends to
spread sequentially from one group of lymph nodes to the next, rarely
skipping lymph nodes; this is a characteristic attribute of this form of
lymphoid cancer.
What are the most common types of Hodgkin lymphoma?
The different types and subtypes of HL are distinguished according to
what they look like under a microscope. The type of tumor a patient
has may affect the choice of treatment. The 2 main types of HL are:
n
n
Classical HL, which affects 95% of patients diagnosed with HL in
developed countries.
Nodular lymphocyte predominant HL.
Classical HL (CHL) is subdivided into the following 4 different
subtypes:
Nodular
Sclerosis
CHL
n
n
n
n
Mixed
Cellularity
CHL
n
n
n
n
9
This is the most common subtype of HL, affecting between
60-80% of patients with the disease.
Under the microscope, the involved lymph nodes often contain
prominent scar tissue—leading to the name nodular sclerosis
(sclerosis means scarring).
The disease is equally common in men and women, and it
usually affects teenagers and young adults.
Most patients are cured with current treatments.
This type of CHL affects 15-30% of patients with HL and is found
more commonly in men than women.
Under the microscope, the lymph nodes contain many R-S cells
mixed with many other types of cells.
This disease primarily affects older adults.
More extensive disease is usually present by the time patients
with this subtype are diagnosed.
Understanding Hodgkin Lymphoma
LymphocyteRich CHL
n
n
LymphocyteDepleted CHL
n
n
n
Under the microscope, this subtype shows many normal
lymphocytes and very few abnormal cells and R-S cells.
This disease is usually diagnosed at an early stage in adults and
has a low rate of relapse or recurrence.
This is the least common form of HL, accounting for fewer than
5% of all patients diagnosed with the disease.
Under the microscope, there are very few normal lymphocytes
and many R-S cells.
This is an aggressive lymphoma that is usually not diagnosed
until the disease is widespread.
Nodular lymphocyte predominant HL is rare, affecting 5-10% of
patients diagnosed with HL. It affects more men than women and is
usually diagnosed in people under 35. This subtype is often found in
the neck lymph nodes, is usually diagnosed at an early stage, and it is
not very aggressive (it grows slowly). In many ways, this form of HL is
similar to low-grade (indolent) B-cell NHL.
Why do some people develop Hodgkin lymphoma?
The reasons why people develop HL are not well understood.
However, scientists have found that people with certain characteristics
have a higher risk of developing HL compared with people who do not
have these characteristics.
The characteristics that make a person possibly more susceptible to
developing any type of disease are called risk factors. Having 1 or
more of these risk factors does not mean a person will develop HL.
In fact, most people with risk factors never develop the disease and
many people diagnosed with HL have never been exposed to clearly
identifiable risk factors.
Learning the Basics
10
Part 1
n
This subtype of CHL occurs in 5% of all patients diagnosed with
HL.
Known risk factors for HL include:
Age
Family
History
n
n
n
Sex
Infection
by Certain
Viruses
Weakened
Immune
System
n
n
n
People who are 15-35 years or over 55 years have a higher risk
of developing HL than people in other age groups.
People with a family history of the disease, especially among
first-degree relatives (parent, brother, or sister), have a higher
risk of developing the disease compared with people who do not
have first-degree relatives with HL; siblings of patients with HL
have a 3- to 7-fold increased risk of developing this disease, and
the risk is higher in identical twins. Because HL is a relatively
rare cancer, even with a 3- to 7-fold increase in risk, the chance
of a patient’s family member developing HL is still very low.
Only 1% of patients with HL have a family history of the disease.
Men have a slightly higher risk than women of developing HL,
but more women than men have the nodular sclerosis subtype.
People infected with Epstein-Barr virus (EBV; which causes
mononucleosis) or the human immunodeficiency virus (HIV;
which causes AIDS) have a higher risk of developing HL
compared with people who do not have these infections.
People who have a weakened immune system because of an
inherited condition or the use of immunosuppressant drugs
to prevent organ transplant rejection have a higher risk of
developing HL compared with people who have a healthy
immune system.
HL cannot be caused by injury or by catching it from someone who
has the disease.
11
Understanding Hodgkin Lymphoma
Part 1 — Learning the Basics
Chapter 2: Seeking Medical Help
Part 1
This chapter explains the signs and symptoms of HL and discusses
how a doctor determines whether or not a person has the disease.
A symptom is anything unusual in a normal body function,
appearance, or sensation that a patient experiences. During a visit
with a healthcare practitioner, patients should report all of their
symptoms to their doctor or nurse. Symptoms may indicate the
presence of lymphoma or another disease.
Signs are anything unusual that doctors or nurses notice when they
examine their patients.
What are the signs and symptoms of Hodgkin lymphoma?
The following are signs and symptoms of HL. Keep in mind that 3 out
of 4 people (75%) diagnosed with HL do not have any symptoms, and
that none of these are specific to HL exclusively.
n
Lumps under the skin in the neck, under the arm, or in the groin.
These lumps are swollen lymph nodes that are usually not tender or
painful.
– Infections, not lymphomas or other cancer, are the most common
reasons for swollen lymph nodes; in these cases the swollen
lumps should return to normal size within a few weeks or months
after the infection goes away. For patients with HL, these lumps
persist, although they may fluctuate in size (i.e., get smaller).
Learning the Basics
12
n
n
n
Unexplained intermittent fevers that may last for many days or
weeks.
Unexplained itching that grows steadily more severe over time and
usually affects the trunk, arms, and legs, but it can also be more
localized.
Weight loss (usually by more than 10% of a person’s normal
weight).
n
Extreme sweating at night—enough to drench sheets.
n
Lack of energy.
n
Coughing, shortness of breath, or chest discomfort may be signs of
HL in the chest.
Having one or more of these symptoms does not mean that a person
has HL. These symptoms may be caused by an infection or other
conditions (including other cancers).
When should a patient seek medical attention?
Anyone who has persistent symptoms should see a doctor to make
sure that lymphoma or another serious condition is not present. A
good rule of thumb is to seek medical attention if any of the previously
mentioned symptoms last longer than 2 weeks or sooner depending
on their severity and impact on a person’s daily life. Many of these
symptoms can arise from different causes and need not be cancer.
There are not any specific tests that doctors can use to routinely
screen patients to see if they have HL. During the visit with the doctor,
patients should describe all of their symptoms. The doctor will ask
detailed questions about medical history and perform a complete
physical examination. During the physical examination, the doctor will:
n
n
13
Check for swollen lymph nodes under the chin, in the neck and
tonsil area, above the shoulders, on the elbows, in the armpits, and
in the groin.
Examine other parts of the body to see if there is swelling or fluid in
the chest or abdomen that may be caused by swollen lymph nodes.
Understanding Hodgkin Lymphoma
n
n
Ask about any pain experienced.
Part 1
n
Examine the abdomen to see whether any internal organs are
enlarged.
Look for any weakness or paralysis that may be caused by an
enlarged lymph node pressing against nerves or the spinal cord.
If doctors suspect lymphoma after reviewing the symptoms reported
and signs they have uncovered during the examination, they will order
other tests to confirm the diagnosis.
These tests should include a biopsy and may also include blood tests,
chest x-rays and other imaging tests, scans, and a bone marrow
evaluation. These tests and procedures are discussed in more detail
in the next chapter.
Learning the Basics
14
Part 1 — Learning the Basics
Chapter 3: Getting a Diagnosis
Doctors need the results of different diagnostic tests to accurately
determine whether or not a patient has HL. This chapter explains the
purpose of these different tests and describes what to expect during
and after these procedures.
Cautions About Interpreting Diagnostic Reports
n
n
n
n
n
n
n
Only a biopsy is definitive.
Tests can be reported as “normal” even though lymphoma may
be present.
Tests may be reported as “abnormal” even though lymphoma is
not present.
Other conditions may mimic HL.
The interpretation of tests, such as imaging studies and scans,
can be difficult in some situations and needs to be made in the
context of the disease and the patient.
Often, follow-up tests are needed to determine the true
significance of previous results; additional biopsies may be
needed to clarify the results.
Some patients like to review their written scan reports; when
doing so, it is important to carefully review the findings with their
doctor.
How is Hodgkin lymphoma diagnosed?
The only way to be absolutely sure of a diagnosis of HL (or any
cancer) is for a doctor to perform an excisional biopsy to remove an
entire lymph node or an incisional biopsy to remove a portion of the
diseased tissue (see next page, “What is a biopsy?”). A pathologist
will examine slides containing portions of the biopsy sample under a
15
Understanding Hodgkin Lymphoma
microscope to see if it contains the characteristic features of ReedSternberg (R-S) cells and to see if the cells are arranged in ways
typical of HL.
A core needle biopsy is sometimes used to establish a primary
diagnosis, but is not optimal due to the small size of the biopsy
sample. In situations where the site of biopsy is difficult to access, a
core needle biopsy may be reasonable for diagnostic purposes (see
the chart on the next page).
In addition to routine pathology analyses, portions of biopsy samples
will be used for other tests to confirm the diagnosis and to more
exactly identify the specific subtype of HL. This chapter explains how
these tests work and what kind of information they provide.
What is a biopsy?
A biopsy is a procedure in which a piece of tissue from an area of
suspected disease is removed from the body and examined under a
microscope. The information provided by this tissue sample is crucial
to correctly diagnose the disease and decide on the best course of
treatment.
Learning the Basics
16
Part 1
A surgical pathologist is a doctor who specializes in the diagnosis of
diseases by studying the cells from a patient’s blood, body fluids, and
tissue samples. A hematopathologist specializes in the pathology of
blood cells, and a lymphoma pathologist specializes in the diagnosis
and classification of HLs and NHLs. These doctors are trained to
recognize different cell types by looking at the shape and size of cells
and how they are grouped inside a tissue.
The following are the 2 main types of biopsies doctors use for the
initial diagnosis of patients with a lymphoma:
Excisional
or Incisional
Biopsy
n
n
n
Core
Needle
Biopsy
n
n
n
n
This is the preferred type of biopsy to establish an initial diagnosis
of lymphoma because it allows for the removal of bigger samples
than other biopsy procedures. The larger the sample, the more
tissue the pathologist can examine, which improves the accuracy
of diagnosis.
In this procedure, a surgeon cuts through the skin to remove an
entire lymph node (excisional biopsy) or a large portion of tissue
(incisional biopsy).
If the lymph node is close to the skin surface, the procedure can
be done under local anesthesia to numb the area. If the lymph
node is in the chest or abdomen, the patient is sedated and the
surgeon removes the tissue, usually by making a small incision
above the sternum (breast bone).
This procedure is used when the lymph nodes are deep in the
chest or abdomen or in other locations that are difficult to reach
with excisional biopsy, or when there is a medical reason for
avoiding an excisional or incisional biopsy.
In this procedure, a large needle is inserted into a lymph
node suspected to be cancerous and a small tissue sample is
withdrawn.
A needle biopsy can be done under local anesthesia and stitches
are usually not required.
Sometimes the material collected may not be adequate for
diagnosis and a subsequent excisional or incisional biopsy may
be necessary.
A fine needle aspirate (FNA) biopsy is, as the name implies, a type of
biopsy performed with a very thin needle (smaller than that used for
a core needle biopsy). Because of the small needle size, the sample
will only contain scattered cells without preserving how the cells are
actually arranged in the lymph node. This limited information is not
enough for a precise diagnosis. A FNA biopsy is most often used to
check for return of the disease (relapse) and is virtually never used for
the initial diagnosis.
17
Understanding Hodgkin Lymphoma
What questions should a patient ask the doctor before
having a biopsy or other diagnostic procedures?
Patients diagnosed with a complicated disease will be asked to
undergo a variety of procedures for the initial diagnosis and work-up
before treatment begins, during the course of treatment, and during
the follow-up period. Before patients agree to a procedure, they should
make sure that they understand the reasons for the procedure and
what will be involved. Here is a list of questions patients may want to
ask their doctor.
n
Why is this procedure necessary?
n
What will the procedure tell us about my condition?
n
Can the same information be obtained in another way?
n
What is involved in doing this procedure?
n
What are the possible risks, complications, and side effects?
n
Where will I have the procedure done?
n
Will I have to do anything to prepare for the procedure?
n
How long will the procedure take? Will I be awake? Will I feel pain?
n
How long will it take for me to recover from the procedure?
n
Should anyone else be present when I have the procedure?
n
Will I need someone to take me home afterward?
n
When will I know the results?
n
When will we talk about the results?
n
What will be my out-of-pocket costs?
Learning the Basics
18
Part 1
After a tissue sample has been removed, it is examined by a
pathologist. If the pathologist’s interpretation of the biopsy is uncertain,
the results should be reviewed by a hematopathologist or preferably
a lymphoma pathologist because a pathologic diagnosis of lymphoma
can sometimes be difficult to make. Arming the medical oncologist or
hematologist with an accurate diagnosis on which sound treatment
decisions can be made is of paramount importance.
What is immunophenotyping?
Immunophenotyping is a process used during evaluation of biopsy
material to distinguish between different types of cells (for example,
between normal lymphocytes and lymphoma cells), by detecting
specific molecules (cell “markers” or “antigens”) found on the cell
surface. These cell markers are detected using special antibodies
grown and chemically modified in the laboratory so that they will
change color when they stick to their corresponding markers.
IMMUNOPHENOTYPING
Antibodies change color upon binding to specific cell markers.
Antibody
19
Antigen
Understanding Hodgkin Lymphoma
This color change is studied under a microscope using
immunohistochemistry (IHC) analysis.
n
n
n
In this test, thin slices of the biopsy sample (or
thin layers of fluid or blood) are placed on slides
and treated with sets of antibodies that recognize
different markers found in different types of
lymphoma cells and normal lymphocytes.
Part 1
Immunohistochemistry
(IHC)
The pathologist examines the slides under a
microscope to look for the visible color change that
happens when the antibody sticks to the marker.
The pathologist identifies and counts the number
of cells that change color (meaning that they are
positive for the marker) with each of the different
antibodies and uses that information to identify the
specific type of lymphoma.
Learning the Basics
20
Part 1 — Learning the Basics
Chapter 4: Work-up Before Treatment Can Begin
After the initial diagnosis of HL, the doctor may order other tests such
as blood tests, bone marrow biopsy and aspiration, imaging studies
(especially x-ray, CT, and PET scans), and heart and lung function
tests. This process is often called the work-up. Some of these work-up
studies are needed to see if and how much the disease has spread to
other parts of the body. Doctors will use these test results to determine
the stage of a patient’s disease. Other tests will check how the disease
has affected a patient’s overall health and major organ functions.
Together all of these tests will provide the information needed to help
patients and their doctors decide on the course of treatment that will
give patients the best chance for curing the disease or putting it into
a lasting remission. This chapter will help you understand how HL is
staged, the reason for the various tests, how these tests work, and
what to expect.
How is Hodgkin lymphoma staged?
Staging is used to describe how widely the lymphoma has spread in
patients with HL. As shown in the following figure, there are 4 main
stages of lymphoma, designated by the Roman numerals I through IV.
This staging system divides the body in half using the diaphragm, the
muscle that divides the chest cavity from the abdominal cavity.
21
Understanding Hodgkin Lymphoma
STAGING OF HODGKIN LYMPHOMA
Stage II:
• Two or more
lymph node
regions near
each other
• One or more
lymph nodes
and one organ
near each other
Stage III:
• Two or more
lymph node
regions in
different parts
of the body
with or without
involvement
of a nearby
organ or the
spleen
Stage IV:
• Widespread
disease
• Multiple organs
• With or without
lymph node
involvement
Your doctor may also add any of the following single letters to the
stage:
n
n
n
A — if patients have not experienced symptoms such as significant
weight loss (more than 10% in 6 months), drenching night sweats,
or fevers.
B — if patients have lost weight (more than 10% in 6 months) and
have experienced drenching night sweats or fevers.
X — if patients have a tumor in the chest that according to a chest
x-ray is at least one-third as wide as the chest, or if tumors in other
areas that are at least 10 cm (4 inches) wide, it is called bulky
disease. Patients with bulky disease usually need more intensive
treatment than patients without bulky disease.
Learning the Basics
22
Part 1
Stage I:
• Localized
disease
• Single lymph
node region
• Single organ
outside lymph
nodes
Remember that advanced stages of HL are curable, unlike advanced
stages of solid tumor cancers, which are more difficult to treat.
What is the purpose of blood tests?
Doctors will test a patient’s blood to measure the quantity of the
different types of cells (red blood cells, white blood cells, and
platelets). While HL cells are not found in the blood, blood tests will
help doctors determine how advanced the disease is and whether
a patient will be able to tolerate certain kinds of treatments. The
blood tests will most likely include: a complete blood count (CBC),
differential (to measure the relative amounts of different types of
white blood cells), platelets, erythrocyte sedimentation rate (ESR),
alkaline phosphatase and other liver enzymes, and serum lactate
dehydrogenase (LDH).
Patients with risk factors for human immunodeficiency virus (HIV) will
also be tested for HIV. Prior to therapy, the blood will also be tested for
exposure to the hepatitis B virus (HBV).
If the patient is a woman of childbearing age, her doctor will also do a
pregnancy test because some of the possible treatments may hurt a
fetus.
The results from these tests will help patients and their doctors decide
between different types of treatments. Many of these blood tests will
be repeated during the course of treatment to check how the treatment
is affecting the patient’s body functions.
What types of imaging tests may be used?
The imaging tests usually recommended for a patient with HL are a
chest x-ray and PET/CT scans of the neck, chest, abdomen, and pelvis.
The purpose of these tests is to help find areas of the body where
there may be cancer, to learn how far the cancer has spread, and later
on to check how well the treatment is working. Most of these tests are
painless, and no anesthetic is required.
23
Understanding Hodgkin Lymphoma
X-ray
n
Computer
Tomography
(CT) Scan
n
n
n
n
Positron
Emission
Tomography
(PET) Scan
n
n
n
n
Findings on a chest x-ray may indicate whether the disease is
“bulky.”
A CT scan takes x-rays from many different angles around the
body. A computer combines the pictures obtained from these
different angles to give a detailed image of organs inside the
body.
Patients with HL often have CT scans of the neck, chest,
abdomen, and pelvis. These tests are useful in determining
how many nodes are involved, how large they are, and
whether internal organs are affected by the disease.
The amount of radiation exposure during a CT scan varies
depending on the area scanned. Most CT scans confer little
risk to the patient, although CT scans of the abdomen and
pelvis do add a moderate amount of risk (1 in 500-1,000) to
the general lifetime risk of cancer.
Before a CT scan, the patient may be asked to drink a
contrast liquid and/or get an intravenous injection of a
contrast dye that will more clearly outline abnormal areas that
may be present in the body.
PET scans are very important for the initial staging of HL.
To perform the test, radioactive sugar is first injected into
the body. Increased uptake of the sugar in areas affected
by HL are detected using a positron camera. The amount of
radiation used for a PET scan is low and poses little risk to a
patient.
PET scans can help determine how much disease is present
(staging) and how well it has responded to treatment.
While CT scans show the size of a lymph node, PET scans
show if the lymph node is active (still has significant disease).
Today, CT and PET scans are often combined into 1 test
(integrated PET/CT).
Learning the Basics
24
Part 1
n
X-rays use radiation to take pictures of areas inside the body.
The amount of radiation used in most diagnostic tests is so
small that it poses little risk to the patient.
Magnetic
Resonance
Imaging (MRI)
n
n
n
Like a CT scan, an MRI takes images from different angles
around the body, but an MRI does not use x-rays like a CT
scan; instead it uses magnets and radiofrequency waves.
MRI is not used routinely for staging, but sometimes can
provide useful additional information.
An MRI can provide important information about tissues and
organs, particularly the nervous system, that is not available
from other imaging techniques. To create a clearer picture, a
contrast dye may be injected into a patient’s vein.
A doctor may order an MRI to get clear images of the bones,
brain, and spinal cord to see if the cancer has spread to these
areas.
What is a MUGA scan?
A MUGA scan (multi-gated acquisition scan) is an imaging test that
looks at how well the heart muscle is working. A MUGA scan is done
to make sure that the patient’s body can withstand treatment with
certain lymphoma drugs that may damage the heart in rare cases.
MUGA scans may be done when patients are resting or exercising,
depending on what their doctor wants to assess.
A doctor will most likely order a MUGA scan if he or she is considering
treating a patient with the drug doxorubicin (Adriamycin). This test is
needed to make sure that the heart is functioning normally because
doxorubicin may be associated with cardiac toxicity. A 2-dimensional
echocardiogram (ECHO) is sometimes used instead of the MUGA
scan to test heart function.
Why might a patient need to have a lung function test?
Lung function tests are done to make sure that the body can withstand
treatment with certain lymphoma drugs that may stress a patient’s
lung function. Lung function tests are usually needed in patients with
HL who will be undergoing treatment with a chemotherapy regimen
that contains the drug bleomycin (Blenoxane). Bleomycin can cause
lung damage. A doctor may order breathing tests before beginning
treatment and at other times during treatment to make sure that a
patient’s lungs are still working properly.
25
Understanding Hodgkin Lymphoma
What is a bone marrow biopsy and aspiration?
What happens during bone marrow biopsy and aspiration?
n
n
n
n
n
The patient lies on the exam table, either on a side or on the
stomach.
For the aspiration part of this procedure, the doctor cleans and
numbs the skin over the hip and inserts a thin hollow needle into the
bone.
The doctor uses a syringe to remove a small amount of liquid from
the bone marrow. Even with the numbing local anesthetic, this
procedure can be painful for a few seconds while the marrow is
withdrawn.
For the biopsy part of this procedure (which is usually done right
after the aspiration), the doctor inserts a slightly larger needle to
take out a small piece of bone and marrow. This procedure may
cause mild pain or a pressure sensation. The procedure does not
require any stitches.
A pathologist looks at the samples under a microscope to see if
there are any signs of HL.
Patients who are anxious about the test should talk with their doctor
and nurse to see whether taking a calming medication before the
procedure would be helpful.
Learning the Basics
26
Part 1
Once the diagnosis of HL is made, the doctor may order a bone
marrow biopsy and aspiration to see if the lymphoma has spread to
the bone marrow. The bone marrow is the spongy, soft material found
inside our bones. Bone marrow biopsy is recommended for patients
with symptoms or stage III or IV disease. The information from this
procedure will help the doctor stage the disease.
Part 2 — Treatment of Hodgkin Lymphoma
Chapter 5: What You Should Know Before Starting Treatment
Getting a cancer diagnosis is an overwhelming experience. It is
perfectly normal to be shocked by the diagnosis, anxious about the
future, and confused about the medical information and decisions that
need to be made. This chapter will help you prepare for the start of
treatment by explaining the next steps and providing tips for talking
with a patient’s doctor about any questions and concerns.
First Steps to Take After Receiving a Diagnosis
n
n
Take care of yourself (eat, sleep, rest, and exercise).
Seek the support of family, friends, and others on whom you
trust and rely.
n
Learn about the disease and treatment options.
n
Find medical care that meets your needs.
n
Find emotional and social support.
n
Understand the cost of care and what your insurance will cover.
n
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Maintain a copy of your medical records (paperwork, test
results, and your own notes).
Who will plan and carry out the treatment?
The treatment of patients with HL is usually overseen by a medical
oncologist or hematologist who specializes in the treatment of patients
with lymphoma. Depending on the patient’s healthcare needs, the
doctor may refer him or her to, or work with, other specialists such as
a radiation oncologist. The patient’s healthcare team will also include
other healthcare professionals such as an oncology nurse, nurse
practitioner, physician assistant, social worker, and registered dietitian.
The healthcare team should work together and communicate with you
to plan, carry out, and monitor the treatment.
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Understanding Hodgkin Lymphoma
What is a prognosis?
Prognosis is the medical term doctors use for predicting how the
disease will progress and the likelihood for recovery. This is often one
of the first things that patients ask their doctor. Keep in mind that HL is
highly curable. About 80% of all patients with HL will be cured of their
disease; that number rises to more than 90% in patients with earlystage disease.
Part 2
Prognosis is usually based on information gathered from hundreds
or thousands of other patients who have had the same disease. This
statistical information provides doctors with a general idea of what to
expect when a patient is diagnosed with a specific type of HL, and
also gives guidance on the kinds of treatments that have been most
successful in treating that cancer.
Keep in mind that no two patients are alike and that statistics
from large groups of people cannot accurately predict what
will happen to a specific patient. The doctor most familiar with
the patient’s situation is in the best position to help interpret these
statistics and understand if and how they may apply to a patient’s
particular situation.
Treatment of Hodgkin Lymphoma
28
Patients with classical HL are grouped in the following prognostic
categories to help doctors decide on the best course of treatment.
Prognosis
Stage
Risk Factors
Early Favorable
Stage I or II
None
Early Unfavorable
Stage I or II
One or more of the following:
n
n
n
n
n
Advanced Favorable: Stage III or
0-1 Risk Factors
Stage IV
Intermediate:
2-3 Risk Factors
Understanding Hodgkin Lymphoma
A high sedimentation rate—also called
erythrocyte sedimentation rate (ESR),
which refers to the distance red blood
cells travel in 1 hour in a sample of blood
as they settle to the bottom of a test tube;
inflammation, infection, cancer, rheumatic
disease, and diseases of the blood and
bone marrow increase the ESR.
Cancer in 3 or more nodal areas.
The presence of B symptoms (fever,
weight loss, or night sweats).
Male.
n
Age 45 years or older.
n
Stage IV disease.
n
n
n
29
Cancer in an organ outside the lymphatic
system but adjacent to a lymph node
containing a tumor.
n
n
Unfavorable:
≥4 Risk Factors
A tumor in the chest larger than onethird of the width of the chest according
to a chest x-ray or at least 10 cm (bulky
disease).
Low blood albumin (protein) level
(below 4).
Low hemoglobin level (below 10.5).
High white blood cell count (15,000
or higher).
Low lymphocyte count (below 600 or less
than 8% of the white blood cell count).
How does having a bulky tumor affect the patient’s
prognosis?
While smaller tumors are usually easier to eliminate than larger
tumors, patients with bulky HL also have a high chance of being cured.
How will we decide what is the best treatment?
There are many effective treatment options for patients with HL.
To identify which treatments may work best, doctors consider the
following:
n
The type of HL.
n
The extent of the disease (stage, location, and bulkiness).
n
Results of blood tests and other lab tests.
n
The presence or absence of lymphoma symptoms.
n
A patient’s overall health.
n
A patient’s age and medical history.
n
A patient’s preferences.
A doctor will discuss the risks, benefits, and side effects associated
with the different treatment choices applicable to the patient’s
particular situation. Share questions and concerns with the doctor so
that together you can decide which option is best. Use the following
questions to help you make an informed decision.
Treatment of Hodgkin Lymphoma
30
Part 2
Having a bulky or high-bulk tumor means that the patient has a tumor
in the chest that is at least one-third as wide as the chest according
to a chest x-ray, or a single mass of tumor tissue greater than 10
centimeters (4 inches) located anywhere. Because of the presence
of bulky tumor, the patient will have to be treated more aggressively
than a patient who has the same stage of disease but no bulky tumor.
Often, but not always, radiation therapy is incorporated into the
treatment of patients with bulky tumors.
Questions to Ask Before Treatment Begins
n
n
n
n
What is the stage of my disease? Where are the tumors?
What are my treatment choices? Which do you recommend for
me? Why?
Are new treatments being studied? Would a clinical trial be
appropriate for me?
n
Do I need more than one type of treatment?
n
What is the goal of treatment?
n
n
n
n
31
What is my exact diagnosis? May I have a copy of the report
from the pathologist?
What are the expected benefits of each type of treatment? How
will we know if the treatment is working? What tests will I need
to have to check if it works? How often will I need to get tested?
What are the risks and possible side effects of each treatment?
Can these side effects be prevented or controlled?
What should I do to take care of myself during treatment?
Are there any late or long-term side effects that I should be
aware of?
n
How long will the treatment last?
n
What are the chances that the treatment will be successful?
n
How will the treatment affect my normal activities?
n
How often will I need a checkup?
n
How much will the treatment cost? Will my insurance cover it?
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When should a patient get a second opinion?
Getting a Second Opinion
n
n
n
Some hematologists/oncologists/lymphoma specialists
associated with medical schools or cancer centers may provide
a consultation and be willing to work together with a local
oncologist to provide treatment and follow-up care.
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As part of the second opinion, another pathologist must review
the tissue and blood samples to confirm the diagnosis. Ask
your doctor about finding a pathologist with a lot of experience
diagnosing patients with lymphoma.
To get a second opinion, you will have to provide the consulting
doctor a complete copy of all medical records, original x-rays,
pathology materials, scans, and reports. When you set up the
appointment, ask their office for a list of all the materials they
will need. It may be useful to keep your own copy of all these
records in case you have questions or concerns later on.
Treatment of Hodgkin Lymphoma
Part 2
Before starting any type of treatment, a patient may consider getting
a second opinion—especially if some characteristics of the diagnosis
are complicated or uncertain. The purpose of the second opinion
is not to question the doctor’s expertise, but to make sure that the
suggested treatment plan is reasonable and optimal for the patient’s
particular case. Do not be concerned that you will offend your doctor
by requesting a second opinion. Most doctors will be supportive and
helpful if you tell them that you would like to get a second opinion.
Ask the doctor if it would be okay to briefly delay the start of treatment
to give extra time to get a second opinion. Keep in mind that some
insurance programs require second opinions; others may cover it if a
patient or doctor requests it.
32
To identify lymphoma specialists to contact for a second opinion:
n
n
n
n
n
n
Ask your current doctors, family members, other patients,
friends, and coworkers.
Contact the patient referral service at your local hospital and
at the nearest hospital associated with a medical school; many
hospitals have online directories that can be searched to find a
specialist in your area.
Visit the American Society of Clinical Oncology (ASCO) at
www.cancer.net to search their oncologist database.
Visit the American Society of Hematology (ASH) at
www.hematology.org/patients to search for hematologists
with an interest in lymphoma.
Visit the National Cancer Institute (NCI) at
http://cancercenters.cancer.gov/ to identify the nearest
NCI-designated cancer center and call or visit their website to
find out about their lymphoma specialists.
Visit the American Board of Medical Specialists (ABMS) at
www.abms.org and click on, “Is My Doctor Board Certified” to
find out if doctors are board certified in a particular specialty.
How to find an oncologist and treatment center?
A patient’s primary care doctor will probably have referred them to a
specialist—likely a medical oncologist, hematologist, or hematologist/
oncologist. Oncologists are physicians who specialize in diagnosing
and treating patients with cancer. Hematologists are physicians who
specialize in diagnosing and treating patients with disorders of the
blood and lymphatic system. Many physicians are experienced in both
areas, and there is some overlap.
Before agreeing to treatment by a specific specialist and treatment
center, make sure that they will be able to meet all medical and
personal needs. Patients should feel comfortable with the healthcare
team and the quality of the care they provide.
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Understanding Hodgkin Lymphoma
Here are some questions to ask to select the best medical team:
n
n
n
n
n
n
n
n
n
n
n
Is the specialist board certified as a medical oncologist and/or
hematologist? Has he or she passed qualifying examinations by
the American Board of Internal Medicine to certify competency
in these specialties?
How much experience do the specialist and treatment center
have in treating patients with cancer in general, and HL in
particular?
Part 2
n
What are the credentials of the specialist, the other members of
the medical team, and the hospital or cancer center?
How many patients with this disease are being treated here
now?
Does the specialist and/or center participate in clinical trials?
How much time does the specialist spend on research and how
much on the treatment of patients with HL?
Is the specialist a member of the American Society of Clinical
Oncology and/or the American Society of Hematology?
Does the clinic or center have modern surgical facilities and
diagnostic equipment?
Is the specialist or clinic affiliated with any major medical center
or medical school?
What arrangements are made for medical coverage after hours
and on weekends, in case of an emergency?
Is my health insurance accepted at this center? Will the office
file claims for reimbursement and process the paperwork?
What kind of patient resources does the clinic or cancer center
have for patients with my disease?
Patients enrolled in a managed care program may have limited
choices. However, patients have the right to choose another
healthcare team if they are not entirely satisfied with their first
consultation visit. They should talk with other patients about their
experience and ask them if they would recommend their specialist and
healthcare team. Also, patients should share their concerns with their
primary doctor and ask for a referral to a different specialist if they are
not satisfied after their first visit.
Treatment of Hodgkin Lymphoma
34
How to communicate with the healthcare team
Patients can ease their anxieties by establishing open, honest
communication with their doctor and nurse regarding their diagnosis,
and learning about what the prescribed treatment regimen is,
how it works, what tests are involved, and what side effects and
complications may be associated with it.
A good first step is to write down all questions that come to mind.
Before meeting with a doctor or nurse, whether for the first time or for
follow-up visits, consider organizing these questions to bring to the
visit. Put the 2 or 3 most important questions at the top of your list,
since time with doctors or nurses may be limited. But make sure that a
member of the medical team reads all of your questions, because they
may see some that are more important than you realize.
Patients should consider having a family member or close friend
accompany them to the doctor’s office or clinic to help ask questions
and understand and remember answers. A companion could also
help by taking notes during the visit. Some patients bring a recording
device to record the answers. Check with the patient’s doctor before
recording any conversations.
Most oncology nurses are also very well informed about cancer
treatments and are a good source of information on a wide range
of topics. Oncology social workers are also available to assist with
practical and emotional needs from the time of diagnosis and onwards.
Although family members are often very concerned about their loved
one and want information concerning his or her care, confidentiality
rules prohibit doctors from giving out information to anyone without
the patient’s expressed permission. For efficiency, designate 1
family member as the family contact. The patient must remember to
specifically tell the doctor the identity of the primary family contact.
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Understanding Hodgkin Lymphoma
Communicating With Your Doctors
At home
n Keep a journal of your symptoms to help you remember the
details you want to discuss with your doctor during your next
office visit.
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Part 2
Make a list of questions that you want to ask your doctor—but
if the questions are urgent, do not wait for the next visit; call the
doctor’s office to discuss your concerns.
At your next doctor’s visit
Bring your symptom journal and list of questions, and discuss
them with your doctor or nurse.
n
n
n
n
n
n
n
Ask a family member or friend to come with you to provide
emotional support and take notes.
Do not be afraid to ask questions if you do not understand
something. Your doctor will want to know if you are uncertain or
confused, and will be happy to explain things to you.
Clarify who should be contacted for specific sorts of questions,
or for weekend support.
Ask if members of your healthcare team communicate by email.
Before leaving the doctor’s office, make sure that you
understand the next steps in your care.
Ask for written information that you can take home to help you
remember what to do, and learn more about your treatment.
Treatment of Hodgkin Lymphoma
36
What is the purpose of an Advance Healthcare Directive
and Appointing a Healthcare Proxy
Having an Advance Healthcare Directive (a living will) and appointing
a Healthcare Proxy is something that all adults should consider doing
regardless of their healthcare status. This is something for everybody
to think about, not just people with cancer—remember that HL is
highly curable but accidents and other unforeseen circumstances can
happen anytime.
Writing down your wishes for critical medical care in an Advance
Healthcare Directive is a way for you to formally tell your doctor, family
members, and friends about your healthcare preferences and what
special treatments you do or do not want if you were critically ill or
injured and unable to make and communicate your own decisions.
Besides stating medical care instructions, the Advance Healthcare
Directive should also include the name of your Healthcare Proxy,
or decision maker. This person should be someone you believe will
carry out your wishes if you are unable to do so, including any do not
resuscitate (DNR) instructions.
Before writing an Advance Healthcare Directive, it is important that you
understand patients’ rights and laws regarding Advance Healthcare
Directives in your state. Consulting an attorney can provide the legal
information, but patients do not have to use an attorney to write an
advance directive. Make sure that your Advance Healthcare Directive
includes:
n
n
37
Specific instructions on medical care, including the types of
special treatment you do or do not want, such as cardiopulmonary
resuscitation (CPR), artificial respiration, drugs to make the heart
function, kidney dialysis, artificial feeding, and certain surgical
procedures.
Your choice of a healthcare proxy.
Understanding Hodgkin Lymphoma
For more information about Advance Healthcare Directive guidelines
for your state, please visit the “Caring Connections” website of the
National Hospice and Palliative Care Organization, at
www.caringinfo.org.
How to be a self-advocate
The first steps in participating in treatment are to ask questions,
learn about options, and work closely with the doctor. Patients must
be comfortable with their doctor and the approach that they take. If
you are not comfortable, you should openly discuss your concerns.
Confidence in the medical team often leads to confidence in treatment.
If you do not feel that the team is a good match, you should ask for a
referral.
Questions will likely vary depending on the purpose of the meeting
with the oncologist (such as the initial visit to discuss the diagnosis or
a routine visit to monitor a remission). Ask for the timing of office visits,
treatments, and tests. The doctor can help explain what the tests will
look for and define the possible responses and the options for further
care depending on treatment response.
Although each person is different and each response to therapy is
unique, knowing someone who has been through the same treatment
and who may have had similar concerns can be a source of great
comfort for patients. If you are interested in talking to and learning
from people who have had similar experiences, you should ask your
oncologist, hematologist, oncology nurse, or the oncology social
worker about any support groups in your area.
Treatment of Hodgkin Lymphoma
38
Part 2
Being a self-advocate and an active participant in your healthcare can
be a positive experience and may help restore any sense of control
you may have felt was lost following your diagnosis. All patients should
remember that they are partners in their treatment plan. Many patients
feel better when they actively participate in their care.
Before you agree to any tests, you should check with your healthcare
team to determine which costs are covered by insurance and which
are not. You should also not be afraid to talk to your healthcare team
about nonmedical issues, such as transportation, finances, insurance,
and childcare.
Self-Advocacy
n
n
n
n
39
Do not be afraid to ask your doctor or nurses questions about
your care.
Learn more about your specific lymphoma by asking your
doctor for information and visiting reliable websites, such as
the Lymphoma Research Foundation at www.lymphoma.org or
www.focusonhl.org.
Take advantage of counseling, support groups, nutritional
counseling, fitness classes, and other services offered at your
doctor’s office, cancer center, or hospital.
Consider joining the Lymphoma Support Network, a nationwide
buddy program that matches patients and caregivers with
people who had similar experiences. For information about the
program, call (800) 500-9976 or email [email protected].
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Part 2 — Treatment of Hodgkin Lymphoma
Chapter 6: Treatments for Hodgkin Lymphoma
n
n
Hodgkin lymphoma after failure of autologous stem cell transplant
or after failure of at least 2 previous combination chemotherapy
regimens in patients who are not candidates for an autologous stem
cell transplant.
Systemic anaplastic large cell lymphoma after failure of at least 1
previous combination chemotherapy regimen.
Brentuximab vedotin is a monoclonal antibody with an attached
chemotherapy drug, also known as an immunoconjugate. The drug
targets CD30, a molecule found on lymphoma cells, and delivers its
attached chemotherapy drug directly to these cells.
Keep in mind that new therapies may have been approved by the
United States Food and Drug Administration (FDA) since this book
went to print. Read Chapter 10 to learn about new agents under
investigation.
Two additional advances in the last 25 years have dramatically
changed the way patients with Hodgkin lymphoma are treated:
1. The elimination of surgical staging laparotomies, in which a patient’s
spleen was removed to evaluate the extent of disease. This was
replaced with noninvasive clinical staging using computerized
tomography (CT) and positron emission tomography (PET) scans to
capture detailed images of internal organs and spot enlarged lymph
nodes.
Treatment of Hodgkin Lymphoma
40
Part 2
In this chapter, you will learn about the most common therapies
that are currently used in the treatment of patients with HL. One of
the most exciting recent developments is the approval in 2011 of
brentuximab vedotin (Adcetris), the first new drug approved for HL in
almost 35 years. Brentuximab vedotin is currently approved for the
treatment of patients with the following:
2. Limiting the use of radiation therapy to subgroups of patients who
require it, and reducing exposure to radiation therapy in patients
who require it by decreasing the dose of radiation and the size of the
field of radiation (the amount of body radiated).
What words do doctors use to describe the outcome of
treatment?
Primary
Therapy
This is the first therapy that a patient receives. The choice of
primary therapy depends on the pathologic characteristics of the
disease, including the factors described previously in this booklet.
Complete
Remission
(CR)
This term is used when all signs of the lymphoma have disappeared
after treatment. This does not mean that the cancer is completely
cured; it means that the symptoms have disappeared and the
lymphoma cannot be detected using current tests. If this response is
maintained for a long period, it is called a durable remission.
Cure
This word is cautiously used by doctors when there are no signs
of the lymphoma reappearing after many years of continuous,
complete remission. The term is most often applied to diffuse large
B-cell lymphoma or Hodgkin lymphoma.
Disease
This term means that the disease has worsened or the tumor
Progression has grown during therapy. Other terms used to describe this are
treatment resistance or resistant disease.
Partial
Remission
(PR)
This term is used if the lymphoma has responded to treatment and
shrunk to less than one-half of its original size.
Improvement This term is used if the tumor has shrunk following therapy but is
still more than one-half of its original size.
Stable
Disease
This term means that the disease has not gotten worse or better
following therapy (the tumor has not grown or shrunk), but has
stayed about the same size.
Refractory
Disease
This refers to a cancer that is resistant to treatment (meaning that it
has not disappeared after treatment).
Relapse
This term refers to disease that reappears or grows again after a
period of remission.
41
Understanding Hodgkin Lymphoma
What types of treatments are used in patients with classical
Hodgkin lymphoma?
There are 3 general types of therapies for patients with classical HL:
n
n
n
Radiation therapy, which uses high-energy radiation to kill cancer
cells.
Chemotherapy, which affects general cell growth and division.
Part 2
Biological therapy, which works with the body’s normal functions
(such as the immune system) to fight cancer.
These therapy types are described in detail throughout the chapter.
Most people treated for HL receive some form of chemotherapy
and sometimes radiation therapy as their frontline treatment. Bone
marrow or stem cell transplantation is typically used in the relapsed
or refractory setting. Surgery is used to obtain a biopsy for diagnostic
purposes, but it is not a treatment or cure for HL.
Although the cure rate in HL is already high, researchers continue to
look for ways to treat the small minority of patients whose disease is
resistant (refractory) to treatment. For the small number of patients
who relapse, alternative therapies, such as stem cell transplantation,
may cure the cancer. Ongoing research also continues to find ways
to refine currently available treatments by limiting their toxicity and
potential for causing secondary illnesses. Better targeted drugs that do
not cause such damaging side effects are also being pursued.
Treatment of Hodgkin Lymphoma
42
This chart shows how different types of classical HL are usually
treated. PET/CT scans are often used after several cycles of treatment
to determine how many more cycles of treatment are needed.
Stage/Type of Lymphoma
Commonly Used Treatment(s)
Stages IA and IIA
n
n
n
Stages I and II, Bulky
Disease
n
n
n
Stages III and IV
n
n
Resistant Disease or
Recurrent or Relapsed
Disease
n
n
n
Chemotherapy followed by radiation therapy to
the initial site of disease (involved field radiation
therapy, IFRT).
4-6 cycles of chemotherapy alone.
For patients who do not respond to treatment,
high-dose chemotherapy (possibly with radiation
therapy) followed by stem cell transplantation.
4-6 cycles of chemotherapy usually followed by
IFRT.
Chemotherapy alone (typically 6 cycles).
For patients who do not respond to treatment,
high-dose chemotherapy (possibly with radiation
therapy) followed by stem cell transplantation.
Chemotherapy alone (typically 6 cycles), with
possible addition of radiation therapy for patients
with bulky disease. More intensive chemotherapy
may be used if patients have unfavorable features
as described earlier.
For patients who do not respond to treatment,
high-dose chemotherapy (possibly with radiation
therapy) followed by stem cell transplantation.
Clinical trial.
High-dose chemotherapy followed by autologous
or allogeneic stem cell transplantation.
For patients ineligible for transplantation:
– Radiation therapy and/or chemotherapy with
drugs not previously used.
– Treatment with the immunoconjugate
brentuximab vedotin (Adcetris).
43
Understanding Hodgkin Lymphoma
What types of treatments are used in patients with
lymphocyte-predominant Hodgkin lymphoma?
Lymphocyte-predominant HL (LPHL) tends to grow slowly and
sometimes causes a late relapse. Patients with early-stage favorable
LPHL have a better prognosis than those with classical HL. This chart
shows how stages of LPHL are usually treated.
Stage/Type of Lymphoma
Commonly Used Treatment(s)
Stages IA and IIA
n
Stages IB and IIB
n
Stages IIIA and IVA
Stages IIIB and IVB
Recurrent or Relapsed
Disease
IFRT with or without chemotherapy.
Rituximab (Rituxan) with or without chemotherapy
with or without IFRT.
n
Observation.
n
Chemotherapy with or without radiation therapy.
n
Rituximab with or without chemotherapy.
n
Local radiation therapy (palliation only).
n
Chemotherapy with or without radiation therapy.
n
n
n
n
Part 2
n
Involved field radiation therapy (IFRT) or
observation.
Rituximab with or without chemotherapy with or
without radiation therapy.
Clinical trial.
Observation if recurrence is limited and there are
no symptoms.
Chemotherapy or rituximab with or without
chemotherapy.
In some cases HL can transform into a more aggressive type of NHL.
Rapid growth of 1 or more lymph nodes is a clear indication for a
biopsy to see if such a transformation has occurred. If transformation
occurs, treatment of the resulting NHL will be necessary. If a patient
experiences recurrent or relapsed transformed NHL (after having
completed initial treatment for transformed NHL), then high-dose
chemotherapy followed by autologous or allogeneic stem cell
transplantation is recommended.
Treatment of Hodgkin Lymphoma
44
What is chemotherapy?
Chemotherapy drugs work against general characteristics of cancer
cells such as their tendency to grow and multiply very quickly.
Depending on the drug, patients may have to swallow a pill or receive
it through a liquid infused directly into a vein (intravenous infusion
or IV).
During chemotherapy, patients receive the drug, or drugs, 1 or more
times a week for 1 or more weeks, followed by a rest period. This
regular treatment schedule is called a cycle. The length of the rest
period and the number of cycles vary depending on your disease and
the type of drugs used.
Most patients with HL are treated with combination chemotherapy,
meaning 2 or more drugs, instead of a single drug. These
chemotherapy drugs are given in a specific order (or schedule) during
certain days of each treatment cycle—this is called a treatment
regimen. The reason to combine drugs is to increase how effectively
they kill or damage cancer cells.
Oncology nurses are usually responsible for administering the
chemotherapy regimen prescribed by the doctor. Most patients
receive their chemotherapy in an outpatient clinic, hospital outpatient
department, or doctor’s office, but sometimes people have to stay in
the hospital for their treatment.
45
Understanding Hodgkin Lymphoma
Common chemotherapy regimens for classical Hodgkin
lymphoma
Regimen
Abbreviation
Generic Name of Drugs (Brand Name)
Doxorubicin/hydroxydaunorubicin (Adriamycin, Rubex)
Bleomycin (Blenoxane)
Vinblastine (Velban)
Dacarbazine (DTIC)
Stanford V
Doxorubicin/hydroxydaunorubicin (Adriamycin, Rubex)
Vinblastine (Velban)
Mechlorethamine (Mustargen)
Etoposide (VePesid, Etopophos, Toposar)
Vincristine (Oncovin, Vincasar PFS)
Bleomycin (Blenoxane)
Prednisone (Deltasone)
BEACOPP
Bleomycin (Blenoxane)
Etoposide (VePesid, Etopophos, Toposar)
Doxorubicin/hydroxydaunorubicin (Adriamycin, Rubex)
Cyclophosphamide (Cytoxan)
Vincristine (Oncovin, Vincasar PFS)
Procarbazine (Matulane)
Prednisone (Deltasone)
Part 2
ABVD
Chemotherapy regimens for lymphocyte-predominant
Hodgkin lymphoma
A variety of chemotherapy regimens can be used for lymphocytepredominant HL. The choice of which regimen to use is based
on many factors including patient and physician preference.
Communication between the doctor and patient about appropriate
treatment is recommended. Observation is also an option for patients
with neither symptoms nor bulky disease.
Treatment of Hodgkin Lymphoma
46
Regimen
Abbreviation
Generic Name of Drugs (Brand Name)
ABVD
Doxorubicin/hydroxydaunorubicin (Adriamycin, Rubex)
Bleomycin (Blenoxane)
Vinblastine (Velban)
Dacarbazine (DTIC)
CHOP
Cyclophosphamide (Cytoxan)
Doxorubicin/hydroxydaunorubicin (Adriamycin, Rubex)
Vincristine (Oncovin, Vincasar PFS)
Prednisone (Deltasone)
CVP (COP)
Cyclophosphamide (Cytoxan)
Vincristine (Oncovin, Vincasar PFS
Prednisone (Deltasone)
EPOCH
Etoposide (VePesid, Etopophos, Toposar)
Prednisone (Deltasone)
Vincristine (Oncovin, Vincasar PFS)
Cyclophosphamide (Cytoxan)
Doxorubicin/hydroxydaunorubicin (Adriamycin, Rubex)
How is chemotherapy given?
Depending on the regimen, patients will be given chemotherapy in pill
form, as an injection, or as an intravenous (IV) drip through a vein. To
make it easier to give and receive multiple cycles of chemotherapy
by IV, the doctor may insert an IV catheter that will stay in place for a
few weeks or for the duration the chemotherapy treatment. There are
several types of catheters, described in the following chart. Patients
should discuss with their doctor what type of catheter, if any, would be
best for their particular situation.
47
Understanding Hodgkin Lymphoma
Type of
Catheter
Description
Disadvantages
Hickman and Consists of 1-3
Broviac
tubes surgically
inserted through
the subclavian
vein (the vein that
runs underneath
the collar bone) in
the chest wall into
a vein. Six to 12
inches of tubing
remain outside
the skin.
It makes it easy
to draw blood and
give drugs using
standard needles
and without
having to pierce
the skin.
Requires proper care to
reduce the risk of infection
and blockage.
The tubes on the outside
of the body make it more
obvious that a catheter is
in place.
Patients need training and
instructions to learn to
clean and take care of the
external tubes.
Infusaport or A catheter is
Portacath
surgically inserted
through the
subclavian vein
and attached to
a small reservoir
(port) that lies
under the skin.
Nothing is visible
on the outside
except for a bump
on the chest.
Patients do
not have to do
anything to care
for it; once a
month a nurse
keeps it clean by
“flushing” with a
small amount of
injected liquid.
The patient must be
injected through the skin
with a special needle each
time it needs to be used.
Sometimes it is hard to use
it to draw blood samples
because of clogging (due to
a blood clot).
Peripherally
Inserted
Central
Catheter
(PICC line)
A good option
for patients
who only need
to have many
short infusions
or continuous
infusions given
in a hospital or
at home with a
portable pump.
This is more temporary than
the other types of catheters.
A thin, soft plastic
tube is inserted in
a large vein in the
arm.
Treatment of Hodgkin Lymphoma
Part 2
Advantages
48
Why is it important to adhere to the chemotherapy
treatment schedule?
Patients should adhere to their chemotherapy treatment schedule
because a full course of chemotherapy works best in the treatment of
their disease. In clinical studies, doctors have found that reducing the
dose or delaying chemotherapy may decrease the chance of a cure
and long-term survival for patients with certain types of lymphomas.
Changing the regimen to reduce short-term side effects may actually
be harmful in the long run. Some side effects may be unpleasant
but tolerable (see Part 3, “Side Effects and Living With Cancer”).
Other side effects may be serious but can often be anticipated and
prevented. It is very important that chemotherapy schedules be
maintained to the greatest extent possible.
What is radiation therapy?
Radiation therapy (also called radiotherapy) uses high-energy x-rays
or other types of radiation to kill cancer cells and shrink tumors.
Radiation therapy is limited to patients who require it because of the
potential long-term toxicities of this treatment.
A radiation oncologist will be in charge of your radiation therapy. The
part of the body selected to receive the radiation therapy is called the
radiation field. In HL, the common areas of the body that are radiated
include lymph nodes in the neck, chest, and armpit (mantle field);
lymph nodes in the abdomen and possibly spleen; lymph nodes in the
pelvis and groin; and in certain circumstances, extended field radiation
to both the mantle and upper abdominal fields.
Doctors will decide on the type and size of the radiation field
depending on the type of tumor and the extent of disease. The total
dose of radiation is usually divided and given over 1-6 weeks. During
and after the radiation treatment, patients will have to carefully protect
the radiation site from the sun.
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Understanding Hodgkin Lymphoma
External beam radiation therapy is the type of radiation therapy
used to treat patients with HL. This form of radiation therapy uses a
machine outside the body to send electrons, or negatively charged
particles, directly to a specific area of the body. The 2 main types of
external beam radiation therapy used against HL are:
n
Extended field radiation (EFR)—In EFR, the radiation field includes
the lymph node regions that contain HL and the surrounding normal
lymph nodes, in case the disease has spread to levels that cannot
yet be detected. EFR is seldom used in patients with classical
HL because most are also treated with chemotherapy. EFR is
sometimes used as standalone therapy in patients with nodular
lymphocyte-predominant HL.
To prepare for radiation therapy, the healthcare team will precisely
mark the patient’s body with tiny ink dots (called tattoos) to make
sure that only the targeted areas receive radiation. During the day
of treatment, they will use lead shields to protect a patient’s normal
tissues around the radiation field. They use plastic forms, pillows, and
rolled blankets to make the patient comfortable and to keep him or
her in the proper position. Patients need to lie still on a table beneath
a large machine that delivers the radiation painlessly. Once the
preparations have been made, it takes only a few minutes to deliver
the prescribed dose.
Treatment of Hodgkin Lymphoma
50
Part 2
n
Involved field radiation therapy (IFRT)—In IFRT, the radiation field
includes the lymph node regions that are known to contain HL. IFRT
is the preferred form of radiation therapy for patients with HL and is
usually given after chemotherapy. IFRT is used alone only to treat
certain patients with nodular lymphocyte-predominant HL.
Questions to Ask Before Starting
Radiation Therapy
n
What is the goal of my radiation therapy?
n
How will the radiation be given?
n
When will treatment begin? When will it end?
n
How will I feel during the therapy?
n
n
n
What are the side effects of radiation therapy? Is there anything
that can be done to prevent them?
Are there any lasting effects?
What can I do to take care of myself during and after the
therapy?
n
How will we know if the radiation therapy is working?
n
How will the radiation treatment affect my normal activities?
What are complementary and alternative remedies?
Alternative therapy refers to treatments that are used instead of
standard therapy recognized as effective by the medical profession.
Currently, there are no viable alternative therapies to
conventional cancer care for patients with HL. Patients should
not use alternative remedies to replace the standard care
suggested by their doctors.
Complementary therapy is used in addition to standard medicine to
help improve a patient’s quality of life and to relieve the effects of
chemotherapy, radiation, and surgery. Patients should talk to their
doctor before starting any form of complementary therapy because
some of these practices can make cancer treatment less effective.
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Understanding Hodgkin Lymphoma
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Complementary therapy, also known as integrative medicine, includes
the following varied forms of therapy:
Mind/Body
Therapies
n
n
n
n
n
n
Acupuncture
n
n
Nutrition
n
Meditation, guided imagery, and self-hypnosis can help manage
stress.
Yoga and Tai Chi minimize stress and improve balance and
flexibility.
Examples: massage, reflexology (foot massage), Reiki, etc.
Part 2
Touch
Therapies
Examples: meditation, guided imagery, self-hypnosis,Tai Chi,
yoga.
These techniques apply therapeutic pressure to the body to
restore a sense of harmony, relaxation, and well-being.
Studies suggest that massage may lessen pain.
Some studies show that acupuncture may relieve pain, nausea,
fatigue, hot flashes, and neuropathy (numbness and tingling in
the feet and hands) associated with chemotherapy and may help
decrease mild depression.
Using ultra-thin needles applied to specific points on the body,
acupuncture is safe and painless. Needles used should be
disposed of and only used once.
Patients undergoing lymphoma treatment should eat a healthy,
well-balanced diet that contains 5-7 servings of fruits and
vegetables a day, fish or poultry, and whole grains.
Treatment of Hodgkin Lymphoma
52
What is relapsed or refractory Hodgkin lymphoma?
Relapsed HL means that the disease has returned after responding
to treatment—this is sometimes also called a recurrence. Refractory
HL means that the patient’s disease no longer responds to a specific
treatment. Patients with Hodgkin lymphoma whose disease does
not go into complete remission after initial therapy or who relapse
after achieving complete remission are often treated with second-line
chemotherapy regimens, followed by a stem cell transplant if they
respond to second-line treatment.
There are many treatment options for patients with relapsed or
refractory lymphomas. Exactly what type of treatment is optimal for
individual patients with relapsed or refractory lymphoma depends on
factors such as the type of lymphoma, age, extent of disease, overall
health, and previous therapies received. The preferred treatment
for healthy patients with relapsed HL is a chemotherapy regimen
that is typically different from that used initially, followed by highdose chemotherapy with autologous stem cell transplantation–i.e.,
transplantation using the patient’s own stem cells (in the following
pages see, “What is stem cell transplantation?”).
This chart shows examples of second-line chemotherapy regimens
used in patients whose disease has relapsed or recurred after the
initial treatment.
Regimen
Abbreviation
Generic Name of Drugs (Brand Name)
ChlVPP
Chlorambucil (Leukeran)
Vinblastine (Velban)
Procarbazine (Matulane)
Prednisone (Deltasone)
C-MOPP
Cyclophosphamide (Cytoxan)
Vincristine (Oncovin, Vincasar PFS)
Procarbazine (Matulane)
Prednisone (Deltasone)
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Understanding Hodgkin Lymphoma
Dexamethasone (Decadron)
Cisplatin (Platinol, Platinol-AQ)
High-dose Cytarabine (Cytosar, DepoCyt)
ESHAP
Etoposide (VePesid, Etopophos, Toposar)
Methylprednisolone (Medrol)
High-dose Cytarabine (Cytosar, DepoCyt, Tarabine PFS)
Cisplatin (Platinol, Platinol-AQ)
GCD
Gemcitabine (Gemzar)
Carboplatin (Paraplatin)
Dexamethasone (Decadron)
GVD
Gemcitabine (Gemzar)
Vinorelbine (Navelbine)
Liposomal doxorubicin (Doxil)
ICE
Ifosfamide (Ifex)
Carboplatin (Paraplatin)
Etoposide (VePesid, Etopophos, Toposar)
IGEV
Ifosfamide (Ifex)
Gemcitabine (Gemzar)
Vinorelbine (Navelbine)
Prednisone (Deltasone)
Mini-BEAM
Carmustine (BCNU, BiCNU)
Etoposide (VePesid, Etopophos, Toposar)
Cytarabine (Cytosar, DepoCyt, Tarabine PFS)
Melphalan (Alkeran)
MINE
Mesna (Mesnex)
Ifosfamide (Ifex)
Mitoxantrone (Novantrone)
Etoposide (VePesid, Etopophos, Toposar)
VIM-D
Etoposide (VePesid, Etopophos, Toposar)
Ifosfamide (Ifex)
Mitoxantrone (Novantrone)
Dexamethasone (Decadron)
Part 2
DHAP
If the disease responds to second-line therapy, patients with classical
HL should receive high-dose chemotherapy followed by stem cell
transplantation, if eligible.
Treatment of Hodgkin Lymphoma
54
If the disease does not respond to second-line therapy, patients
with classical HL may be treated with radiation therapy, salvage
chemotherapy given with or without radiation therapy, or treatment
with the immunoconjugate brentuximab vedotin (Adcetris), which is
approved for HL after failure of autologous stem cell transplant or after
failure of at least 2 previous chemotherapy regimens in patients who
are not candidates for autologous stem cell transplant.
Patients who do not go into complete remission following treatment
or who do not respond to treatment should not lose hope. Complete
remission may be achieved following a diagnosis of relapsed or
refractory disease. Many patients seek second opinions at any point
from diagnosis onward and often chose to do so if their disease
relapses or is considered refractory. Lymphoma research continually
evolves as doctors and scientists discover new therapies and more
effective ways of giving existing treatments. Chapter 10 describes
some of the options currently under investigation.
What is stem cell transplantation?
There are different types of stem cell transplantation, depending on
who donates the stem cells. In an autologous stem cell transplant, the
patient is the donor. In an allogeneic stem cell transplant, the donor
is another person who is genetically similar to the patient—typically a
brother or sister, but the donor can also be an unrelated person.
The purpose of autologous or full-intensity allogeneic stem cell
transplantation is to allow patients to receive high-dose chemotherapy.
Such high doses effectively kill cancer cells but also severely damage
the bone marrow as a side effect, destroying the body’s source of
blood cells responsible for fighting infection, preventing bleeding, and
carrying oxygen. A stem cell transplantation re-populates the stem
cells responsible for making these blood cells.
Because high-dose therapy and stem cell transplantation place
great strain on a patient’s body, they are not options for everyone.
In deciding if transplantation is a good option, doctors will consider
55
Understanding Hodgkin Lymphoma
the patient’s health status, age, medical history, cancer stage, and
response to previous therapy.
ALLOGENEIC STEM CELL TRANSPLANTATION
Stem cell transplantation using stem cells from a donor who is
genetically similar to the patient.
2. Processing
Blood or
bone marrow
may be processed
in the laboratory
to purify and
concentrate the
stem cells.
Donor
Part 2
1. Collection
Stem cells are
collected from
the donor’s bone
marrow or blood.
3. Infusion
Stem cells
are infused
into the patient.
Patient
AUTOLOGOUS STEM CELL TRANSPLANTATION
Stem cell transplantation using a patient’s own stem cells.
1. Collection
Stem cells are
collected from the
patient’s bone
marrow or blood.
2. Processing
Blood or
bone marrow
may be processed
in the laboratory
to purify and
concentrate the
stem cells. Samples
are frozen until needed.
3. Reinfusion
Stem cells
are thawed and
reinfused
into the patient.
Patient
Treatment of Hodgkin Lymphoma
56
Reduced-intensity transplantation (also called non-myeloablative or
mini-allogeneic transplantation) uses lower doses of chemotherapy
and/or radiation prior to allogeneic transplantation. This option is
available only for allogeneic transplantation and cannot be used for
autologous transplantation. This approach takes advantage of the
graft-versus-disease effect, in which the transplanted cells (the “graft”)
recognize the cancerous cells in the patient’s body as foreign and
destroy them. Patients receiving reduced-intensity transplants may
avoid some of the side effects seen with higher-dose chemotherapy,
although they still have increased risks of serious side effects as
compared with autologous stem cell transplantation due to the
potential for graft-versus-host disease (in which immune cells in the
transplant recognize the patient cells as “foreign” and attack them).
Questions to Ask Before Deciding to Undergo
Stem Cell Transplantation
n
What type of transplantation is most appropriate for me?
n
Why do you think this is a good idea?
n
What are the risks associated with this procedure?
n
Why do you recommend this particular type of transplantation?
n
If I need a donor, how will I find one?
n
How long will I need to be in the hospital?
n
What type of special care will I receive?
n
How sick will this treatment make me?
n
What will you do to lessen the side effects?
n
How will we know if the treatment is working?
n
n
57
How and for how long will the treatment affect my normal
activities?
What is my chance of making a full recovery?
Understanding Hodgkin Lymphoma
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For more information on transplantation, visit the “Be the Match”
website at www.bethematch.org.
Drug costs: What to do if insurance will not pay
Patients in need of financial assistance should talk with their doctor
and social worker about available options and how to enroll in an
appropriate program. Before undergoing a medical procedure, patients
should check with their insurance carrier to ensure that it is covered.
If there is a dispute about coverage, or if coverage is denied, patients
should ask their insurance carrier about its appeals process. Contact
your state’s department of insurance if a claim is denied.
When should a clinical trial be considered?
Clinical trials are appropriate for patients at all stages of disease.
The goal of a clinical trial is to improve the treatment outcome and
minimize side effects as compared with standard treatment. When
treatments studied in clinical trials are successful, they become the
new standard treatments. By participating in a clinical trial, patients
may or may not get access to the newest therapies but will receive
quality care in a very carefully controlled and supportive environment.
Participation in a clinical trial is especially important for patients with
HL because it is a rare disease, which makes it very hard to find
enough patients to enroll in studies, which are critical for improving
treatment. If patients are interested in participating in a clinical study,
they should ask their doctor if there is an appropriate trial for them and
what the potential risks and benefits may be.
Treatment of Hodgkin Lymphoma
58
Part 2
Many patients today face the problem of how to pay for rising
healthcare costs. Cancer organizations like the Lymphoma Research
Foundation (www.lymphoma.org) offer limited financial assistance to
patients who qualify. Most pharmaceutical/biotechnology companies
have patient assistance programs in place that provide drugs for free
to qualifying patients.
Part 3 — Side Effects and Living With Cancer
Chapter 7: Common Treatment Side Effects
Patients with HL may experience various side effects (also called
toxicities) caused by their cancer treatment. Fortunately, medications
and lifestyle changes can effectively prevent or lessen the severities of
most of these side effects. Patients should ask their healthcare team
about possible treatment side effects and how to prevent and manage
them, and tell their doctor or nurse if they experience any side effects.
This chapter explains the causes of these side effects, the types
of side effects caused by different treatments, and steps to take to
minimize these side effects.
Why does chemotherapy cause side effects or toxicities?
Chemotherapy causes side effects because of the non-specific
way these drugs attack cancer cells. Most chemotherapy drugs are
designed to kill cells like cancer cells that divide rapidly (meaning that
they are multiplying quickly). Most normal cells in the body do not
divide as quickly as cancer cells. However, healthy cells in hair roots,
and cells in the mouth, gastrointestinal tract, and the bone marrow do
divide rapidly and can be killed or damaged by chemotherapy. Some
chemotherapy drugs can also damage heart or lung cells.
The type and severity of side effects caused by chemotherapy
vary widely depending on the types of drugs that are given and an
individual patient’s response. The same drug may cause no side
effects in one patient, while in others it may cause anything from very
mild to very serious side effects.
What is the difference between long-term effects and late
effects of treatment?
Long-term effects of treatment are toxicities that happen during
cancer treatment and continue for months or several years. Fatigue,
menopausal symptoms, infertility, and lung problems are examples of
long-term effects.
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Understanding Hodgkin Lymphoma
Late effects of treatment become apparent only after treatment has
ended and may arise many months, years, or even decades after
treatment is completed. Cardiovascular problems, osteoporosis, and
secondary cancers (such as lung cancer) are examples of late effects.
The likelihood of late secondary cancers and cardiovascular toxicities
(heart attacks and strokes) is increased with the use of radiation
therapy, particularly if it is extensive. For this reason, the use and
duration of radiation therapy have been reduced in current treatment
regimens compared with the past.
What side effects are caused by chemotherapy?
Part 3
Note that side effects vary depending on the type of chemotherapy;
additionally, these adverse effects can have causes other than
chemotherapy.
Some of the most common side effects include:
n
Changes in taste.
n
Decreased blood cell production.
n
Increased chance of infections.
n
Fatigue.
n
Hair loss.
n
Loss of appetite.
n
Mouth sores.
n
Nausea and vomiting.
n
Problems with sexual function.
n
Cognitive problems (trouble concentrating, impaired memory).
n
Peripheral neuropathy.
Side Effects and Living With Cancer
60
Changes in taste
Some patients will experience a change in the way foods or beverages
taste. Familiar foods may taste differently (dysgeusia) or the flavors of
foods may not be as strong (hypogeusia). Some patients may also feel
that foods have a metallic taste. These side effects are temporary and
usually disappear after the end of chemotherapy.
Decreased blood cell production
The bone marrow constantly produces red blood cells, white blood
cells, and platelets. Some types of chemotherapy and immunotherapy
temporarily interfere with the ability of the bone marrow to produce
enough of 1 or more of these different types of blood cells. This is
called myelosuppression.
To prevent and control myelosuppression, samples of a patient’s blood
are tested for: complete blood count (CBC), which measures the
number of red blood cells; differential, which measures the amounts
of different types of white blood cells; and the number of platelets.
These tests are usually done before and sometimes during each
chemotherapy cycle.
There are 3 main conditions caused by decreased production of blood
cells:
Anemia
n
n
n
61
This is caused by a decrease in the number of red blood
cells.
Many chemotherapy drugs cause mild or moderate
anemia.
Anemia can make people feel very tired and short of
breath; if needed, anemia can be treated with drugs or
red blood cell transfusions.
Understanding Hodgkin Lymphoma
Neutropenia
n
n
n
n
Thrombocytopenia
n
n
n
Patients with low neutrophil count are at risk of serious
and even life-threatening infections—symptoms of
infection include fever, chills, and night sweats.
A normal white blood cell count ranges from 4,000-10,000
cells per microliter. Doctors regularly monitor the absolute
neutrophil count (ANC), the number of neutrophils in the
peripheral blood. Because patients with an ANC below
500 are at high risk for infections, their doctors may
decrease the chemotherapy dosage or delay the next
treatment in order to keep the ANC above 500.
Some patients require treatment with antibiotics and
hospitalization to prevent or treat infections.
To avoid a patient missing a dose of chemotherapy,
doctors sometimes prescribe drugs like filgrastim
(Neupogen) and pegfilgrastim (Neulasta) to reduce the
duration and severity of neutropenia. These drugs can
sometimes cause bone pain which can be relieved with
nonsteroidal anti-inflammatory drugs (NSAIDs) such as
aspirin and ibuprofen.
Part 3
n
Neutropenia refers to a decrease in neutrophils—the
primary type of white blood cells that fight infection.
Thrombocytopenia refers to a decrease in the number
of platelets in the blood; platelets help start the clotting
process when bleeding occurs.
Patients with low platelet counts may bruise easily; have
cuts that bleed for too long or too much; have nose
bleeds or bleeding gums; or may bleed from places that
have not been injured.
Some patients require a platelet transfusion to stop or
prevent bleeding.
Side Effects and Living With Cancer
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Reducing Your Risk of Serious Infection
During Chemotherapy
n
n
If you have a fever of 100.5°F or greater, call your doctor or go
to the emergency room.
Ask your doctor what you should do if you have a sore throat,
rash, diarrhea, a cough, or redness, swelling, or pain around a
wound.
Fatigue
Fatigue is a common side effect of many types of chemotherapy.
Fatigue should decrease after patients have completed their
lymphoma treatment, but it could take weeks or months until they
return to their normal energy levels.
Coping With Fatigue
n
n
n
n
63
Keep a diary to help you keep track of when you have the most
energy and which activities make you feel tired or give you
energy. Use this information to plan your activities for the times
when you have the most energy.
Ask for help.
Exercise if your doctor says it is okay to do so, but do not
overdo it. Try simple stretching and range-of-motion exercises
or a short walk; these may energize you without tiring you out.
Start slowly and build up to the level that is right for you. Ask
your doctor, nurse, or physical therapist to help you create a
personal exercise plan.
Rest and sleep during therapy are very important, but do not
rest more than you need because it may decrease your energy
levels. An afternoon nap helps many patients feel less tired for
the rest of the day. Other patients cannot sleep at night if they
nap during the day. If you have trouble sleeping, talk to your
healthcare team to find out why and what you can do about it.
Understanding Hodgkin Lymphoma
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Hair loss
For many patients with HL, hair thinning or loss (alopecia) is one of the
most distressing side effects of chemotherapy. This thinning or loss of
hair can occur anywhere in the body, including the scalp, eyebrows,
eyelashes, arms, legs, and pelvis. The amount of hair loss may vary.
If hair loss occurs, it often starts 2 or 3 weeks after the first
chemotherapy treatment. Remember that hair loss caused by
chemotherapy is usually temporary. Hair will probably grow back
after the end of chemotherapy treatments. When the hair first grows
back, it may have a slightly different texture or color than it had before
treatment. Over time, the texture and color often return to normal.
n
After washing it, pat your hair dry instead of rubbing it with
a towel.
n
Brush your hair with a soft-bristle brush or a wide-tooth comb.
n
Do not use curlers and hair dryers.
n
Do not color or perm your hair, or treat it with other chemicals.
n
n
Consider using a wig, scarf, turban, soft cotton hat, or head
wrap. Some health insurance companies cover the cost of
wigs if you have a doctor’s prescription. Check your policy to
see if it covers this cost.
Use a hat or scarf to protect your scalp when you are out in
the sun.
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Part 3
Managing Chemotherapy-Induced Hair Loss
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Mouth sores
Some chemotherapy drugs can make a patient’s mouth become red,
sore, or irritated—this is called mucositis. Some patients undergoing
chemotherapy become more susceptible to viral or fungal infections of
the mouth and throat.
Patients should tell their doctor if they have a sore throat. The doctor
will examine the patient’s throat and may take a swab to send to the
lab to check for infection. Several medications are available to treat
different types of infections. To help decrease chances of infection,
patients should have a complete dental checkup and cleaning before
starting chemotherapy.
Preventing and Caring for Mouth Sores
n
n
n
n
n
n
65
Clean your mouth and teeth. Use a soft-bristle toothbrush,
nonabrasive toothpaste, and lip moisturizer.
Do not use mouthwashes that contain alcohol. Your doctor or
nurse may recommend a mouth rinse.
Do not eat citrus fruits (such as oranges, grapefruit, lemons, or
clementines) or drink citrus juices.
Do not eat spicy foods.
Eat soft foods while you are taking chemotherapy to avoid
bruising your gums and other soft tissues in your mouth.
Do not floss your teeth if your blood counts are low.
Understanding Hodgkin Lymphoma
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Nausea or vomiting
Some chemotherapy drugs can cause nausea or vomiting, usually
on the day chemotherapy is administered but sometimes 1 or 2
days later. The doctor may prescribe a drug that prevents vomiting
(antiemetic) before chemotherapy. Examples of antiemetics include:
aprepitant (Emend), ondansetron (Zofran), granisetron (Kytril),
metoclopramide (Reglan), prochlorperazine (Compazine), and
dolasetron (Anzemet), and a variety of corticosteroids, such as
prednisone (Deltasone). In most cases, these antiemetics are able to
partially or completely prevent nausea and vomiting.
n
n
n
n
n
n
Part 3
Controlling or Minimizing
Nausea and Vomiting
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Before chemotherapy, drink a liquid diet, such as broth,
consommé, or water. Do not drink milk.
Do not eat foods that are too hot, cold, sweet, or spicy.
Eat smaller and more frequent meals instead of fewer large
meals each day.
Avoid strong or offensive smells. Get plenty of fresh air.
Take prescribed antiemetics before chemotherapy to prevent
nausea.
If you vomit, make sure to avoid becoming dehydrated.
Side Effects and Living With Cancer
66
Diarrhea
Some types of chemotherapy cause diarrhea. While most patients do
not experience severe diarrhea, the most important thing to remember
is to avoid dehydration, a loss of body fluids. Call your doctor if you
have bloody diarrhea or fever with diarrhea.
Avoiding Dehydration
From Diarrhea or Vomiting
n
n
n
n
n
n
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Drink plenty of liquids (8 glasses a day) such as water or
electrolyte replacement drinks like Gatorade and Powerade.
Look for the following signs of dehydration: dry mouth or skin,
decreased urine, or feeling dizzy or lightheaded when you
stand up.
Do not drink or eat milk products because they worsen diarrhea.
Do not eat high fiber and other hard to digest foods because
they can worsen diarrhea.
Eat plenty of bananas and other high-potassium food (check
with your doctor or dietitian to make sure that these foods will
not interfere with your chemotherapy or other medications that
you are taking).
Take the medicines that your doctor recommends to control
diarrhea or vomiting (call your doctor if symptoms persist).
Problems with sexual function
Chemotherapy can cause a drop in the sex drive (libido). Usually, a
normal libido returns after treatment is finished. Patients should not be
embarrassed to talk with their doctor about any problems or concerns
they have about changes in their libido.
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Sterility
Since chemotherapy and radiation may damage sperm and egg
cells, it can sometimes cause temporary or permanent sterility, or
the inability to have children, in both men and women. The potential
for developing sterility depends on the treatment type and specific
dose, the number of therapies given, and the patient’s age at the
time of treatment. Patients should speak with their doctor about
fertility preservation (such as treatments to protect the ovaries
or cryopreservation of sperm cells, egg cells, or in vitro-fertilized
embryos) before starting treatment. For more information and
resources about sterility, visit the website for Fertile Hope at
www.fertilehope.org.
Chemotherapy can result in mild cognitive impairment such as trouble
concentrating, impaired memory, or issues with motor control. While
such symptoms can be distressful, they typically disappear over time.
Peripheral neuropathy
Some chemotherapy drugs may also cause damage to the nervous
system called peripheral neuropathy. Nerve damage can cause side
effects such as constipation or a tingling sensation in the fingers and
toes.
Cardiotoxicity
Cardiotoxicity refers to damage to cells in the heart or heart muscle.
Long-term use of certain chemotherapy drugs can cause heart
damage in some patients. Doxorubicin is an example of a drug that is
possibly cardiotoxic.
A person’s history of heart disease, high cholesterol and high blood
pressure, and obesity and lifestyle choices such as smoking and lack
of exercise may increase their chance of developing chemotherapyrelated or radiation-related cardiotoxicity.
Side Effects and Living With Cancer
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Part 3
Cognitive problems
Careful monitoring by the patient’s healthcare team can reduce
the chances of developing cardiotoxicity. Before deciding to treat
patients with a cardiotoxic drug, most doctors will prescribe either an
echocardiogram or a MUGA (multi-gated acquisition) scan to measure
their cardiac function. This will ensure that the chemotherapy dose is
well within the range considered safe for the heart.
Lung toxicity
Damage to the lungs is a serious side effect that can be caused by
bleomycin-containing chemotherapy regimens. Patients should report
any changes in lung function such as cough, chest pain, or shortness
of breath to their doctor, who may decide to monitor their lung health
by regularly performing pulmonary function studies during the course
of chemotherapy.
Other possible side effects
Chemotherapy can also cause other side effects such as cough,
skin rashes, general weakness, sore throat, and loss of balance
or coordination. Patients are sometimes at increased risk for viral
infections, particularly shingles (herpes zoster). Many of these side
effects are temporary, but some could last for an extended period.
Patients should call their doctor if they experience any painful local
rash with or without blisters.
What side effects are caused by radiation therapy?
Radiation therapy itself is painless, but it can cause short-term and
long-term side effects. The side effects caused by radiation therapy
vary depending on the type of radiation, the radiation dose, and the
part of the body treated. Side effects are usually worse when radiation
therapy and chemotherapy are given at the same time.
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Understanding Hodgkin Lymphoma
Examples of Potential Short-Term
Side Effects of Radiation Therapy
n
n
n
n
Radiation to the chest and neck can cause a dry sore throat,
mouth sores, and trouble swallowing.
Radiation to any area may cause hair loss in the area and the
skin may become red, dry, and tender.
Radiation to the pelvis, legs, and torso (the large areas of your
body that contain most of your blood-producing bone marrow)
can decrease levels of red blood cells and white blood cells.
Part 3
n
Radiation to the stomach area can cause nausea, vomiting, and
diarrhea.
Radiation to any part of the body may cause fatigue, which may
progressively worsen during the later weeks of the treatment
cycle.
Examples of Potential Long-Term
Side Effects of Radiation Therapy
n
n
n
n
n
n
The development of new cancers in the radiated parts of the
body.
Cardiovascular damage causing an increased risk of heart
attack and stroke.
Lung damage and problems breathing due to radiation to the
chest.
Thyroid problems (such as fatigue and weight) later in life due
to radiation to the neck.
Headaches, memory loss, personality changes, and trouble
concentrating due to radiation to the brain.
Neck muscle weakness (“neck drop”) causing difficulty lifting
the head, and neck pain or discomfort.
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Dry mouth
Some patients treated with radiation therapy experience a temporary
decrease in saliva production, or dry mouth (xerostomia). People with
dry mouth may have problems swallowing foods or thick liquids. Dry
mouth can also cause food particles to stick to the teeth and gums.
Because saliva helps prevent cavities, doctors may advise patients
to go to the dentist for fluoride treatments before they start radiation
therapy.
Fatigue
The likelihood that patients will experience fatigue depends on their
disease and the specific radiation plan.
Loss of appetite and taste
During radiation treatment, patients might lose their appetite for foods
that they normally like. The loss of appetite and taste are usually
short-term problems. Patients should remember to eat well because
their body needs energy and good nutrition to heal. Eating 4 or 5 small
meals a day may be more comfortable than eating 2 or 3 larger ones.
Patients should ask their healthcare team for information on how to
maintain good nutrition during treatment.
Nausea
Radiation treatment can cause nausea, especially in patients who
have radiation to the abdomen. Not eating (especially sweet, spicy,
or fatty foods) a few hours before radiation therapy may help avoid
nausea. A patient’s doctor may prescribe an anti-nausea (antiemetic)
medication to be taken before each radiation therapy session.
Secondary cancers
The risk of developing secondary cancers from radiation therapy
depends on factors such as the amount of radiation given (i.e., the
dose) and the part of the body treated (i.e., the field). For example,
patients with HL treated for limited disease in the chest are at an
increased risk for developing breast, lung, or thyroid cancer. Patients
with extensive disease in lymph node groups below the diaphragm
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Understanding Hodgkin Lymphoma
are at an increased risk of developing colon cancer and other
gastrointestinal cancers. Less common secondary cancers include
basal cell skin cancer and sarcomas of the bone and soft tissue. All
the data currently available are from when higher doses and larger
fields were used. Current contemporary radiation therapy uses
significantly modified fields and the exact risk of secondary cancers is
unknown.
Most secondary malignancies develop more than 10 years after
completion of treatment and the risk is highest when radiation
therapy is used as part of first-line treatment. Lung cancer and breast
cancer are the most common secondary cancers in patients with HL.
Recommendations include:
n
Annual chest x-ray or chest CT for patients at increased risk for lung
cancer due to chest radiation therapy, alkylating agent therapy, or
smoking history.
Annual breast screening—by mammography and/or magnetic
resonance imaging (MRI)—to start no later than 8-10 years after
the end of therapy or at age 40 (whichever is earliest) for women
who received radiation therapy to the chest or armpit (axilla); these
women should also perform monthly self-breast examination and
undergo yearly breast examination by a healthcare professional.
Cardiovascular damage
Radiation therapy can cause damage to the arteries, most commonly
those in the neck (carotid arteries) and in the heart (coronary arteries).
This increases the risk of stroke and heart attack. Like secondary
cancers, this complication increases after 10 years following neck or
chest radiation therapy. Screening stress coronary artery tests such as
a stress echocardiogram and ultrasound studies of the carotid arteries
are often recommended for patients treated with radiation therapy to
the neck and/or chest over 10 years previously.
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n
Skin reactions
Radiation therapy can cause skin changes to the affected area such
as redness, itchiness, dry and peeling skin, sores or ulcers, swelling,
and puffiness. These skin changes usually decrease and disappear
over a few weeks after the radiation therapy ends. Moist areas, such
as around the mouth, may be more prone to irritation; the doctor may
recommend zinc oxide ointment for these areas. The patient’s skin
could look and feel as if it was sunburned, and it may eventually peel.
Skin Care During and After Radiation Therapy
n
n
n
n
n
n
n
n
n
73
Be gentle with your skin; do not rub, scrub, or scratch.
Use lotions and other skin products that your doctor prescribes
or your nurse suggests.
Do not put anything on your skin that is very hot or cold (such
as heating pads or ice packs).
Take a shower or bathe in lukewarm water; if you bathe, do it
every other day and soak for less than 30 minutes; always use
a mild unscented soap; pat dry your skin; do not wash off the
ink markings needed for radiation therapy.
Check with your doctor or nurse before using bubble bath,
cornstarch, cream, deodorant, hair removers, makeup, oil,
ointment, perfume, powder, and sunscreen.
Wear soft clothes and use soft sheets, such as those made with
cotton.
Do not wear clothes that are tight and do not allow your skin to
breathe.
Make your rooms more humid by placing a bowl of water on the
radiator or using a properly cleaned and maintained humidifier.
Do not sunbathe; protect your skin from the sun every day (use
a broad-rimmed hat, long-sleeved shirt, and long pants or skirt
outside).
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n
n
n
n
Do not use tanning beds.
Do not put adhesive tape or bandages on your skin. Ask your
nurse about ways to bandage without tape.
Ask your doctor or nurse if it is okay to shave the affected area;
shave with an electric razor and do not use pre-shave lotion.
Report to your doctor or nurse any skin changes you notice.
Throat irritation
Easing Throat Irritation
During Radiation Therapy
n
n
n
n
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Eat bland foods that are soft, smooth, and easy to digest,
such as pudding, yogurt, milk shakes, and commercial dietary
supplements.
Take small bites and swallow each bite completely before taking
another.
Try drinking thicker liquids, such as fruit that has been pureed in
a blender; they are easier to swallow than thin liquids.
Avoid citrus fruits, especially juices.
Side Effects and Living With Cancer
Part 3
Radiation therapy to the neck, throat, or chest may cause sore throat,
dry mouth, nausea, or cough. Patients may have difficulty eating or
swallowing, especially toward the end of their treatment regimen.
Patients should tell their doctor if they have problems swallowing.
There are treatments for the discomfort. Difficulty swallowing will
usually go away after a few weeks of treatment.
74
What side effects are caused by steroids?
Cortisone, dexamethasone, prednisone, and other corticosteroid drugs
can cause side effects such as insomnia (the inability to fall asleep),
increased appetite, mood or personality changes, high blood pressure,
and weight gain.
Prednisone can also trigger diabetes in patients prone to that disease
or make diabetes worse in patients who already have the disease.
High doses of steroids can also cause osteoporosis in at-risk
patients. Patients should tell their family and friends that personality
changes may occur during treatment. Patients should avoid making
hasty decisions. If personality changes occur, the doctor should be
informed—the dose may need to be reduced.
What side effects are caused by stem cell transplantation?
Patients treated with high doses of chemotherapy and/or radiation
before undergoing a stem cell transplant are at increased risk for
developing infection, bleeding, and other side effects as described
previously (see, “What side effects are caused by chemotherapy?” and
“What side effects are caused by radiation therapy?”).
Patients receiving high-dose chemotherapy with autologous stem cell
support (autologous stem cell transplantation) are followed carefully
for the first 3-4 weeks because of the risks of mouth sores (mucositis),
infection, anemia (low red blood cell count causing fatigue), and
bleeding. Transfusions and antibiotics, often in the hospital, may be
necessary.
Patients receiving stem cells from a sibling or unrelated donor are also
at risk of developing graft-versus-host disease (GVHD), a condition
where the donated marrow’s immune system attacks the patient’s
tissues. GVHD can happen at any time after the transplant. Drugs can
be used to reduce the risk of developing GVHD or to treat the problem
once it develops.
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More information about stem cell transplantation and its effects can be
found at www.bethematch.org.
When should a patient’s doctor be called?
As a general rule:
n
n
n
Call if the patient experiences a side effect that is unexpected or
lasts longer than expected.
Call if the patient experiences a medical problem—such as high
fever, shortness of breath, prolonged or constant nausea and
vomiting, chest pains, and dizziness—that cannot wait for a regularly
scheduled appointment.
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Part 3
If the patient’s doctor or nurse cannot be reached, go to the hospital
emergency room for a medical assessment and place another call to
the doctor.
Part 3 — Side Effects and Living With Cancer
Chapter 8: Managing Your Life During and After Treatment
This chapter talks about some general issues that patients may
encounter while they live their life during and after treatment.
Coping strategies
Each person’s experience with cancer is different, and how he or she
copes with the physical and emotional impact of having HL is unique
to a patient’s personality and situation. Here are some suggestions for
how to cope with some issues that patients may face.
Maintain
a Strong
Support
System
■
■
■
■
Get Help
for
Depression
■
■
■
■
Deal with
Physical
Changes
■
■
■
■
Maintain
a Healthy
Lifestyle
Set
Reasonable
Goals
77
■
■
■
■
■
■
Communicate your fears and concerns about your disease by talking with your family,
friends, doctors, and counselors.
Writing down your concerns in a journal may also help.
Find a support group or other individuals who are also coping with cancer.
Feeling sad or depressed is not unusual in people living with cancer.
Watch out for signs of depression: sleeping more or less than usual; feeling a lack of
energy; crying; inability to concentrate.
Ask for a referral to a psychiatrist, social worker, psychologist, or counselor who will
help you cope with your feelings through talk therapy, medications, or both.
Find a support group of people who have had similar experiences.
Some patients with cancer feel unattractive because of hair loss and other changes in
appearance caused by their treatment.
Ask your doctor what changes you should expect; plan ahead and buy a wig or head
covering if hair loss is a possibility.
Get advice from a beautician about makeup for the areas that you consider a problem.
Ask your healthcare team for advice on how to manage temporary changes such as
dry skin, brittle nails, and a blotchy complexion.
Eat a healthy diet that includes fruits, vegetables, protein, and whole grain.
Engage in regular physical exercise; this can reduce anxiety, depression, and fatigue,
and improve mood.
Get sufficient rest to help combat the stress and fatigue of your disease and its
treatment.
Quit smoking and reduce alcohol consumption.
Having goals for how you want to live your life during and after treatment will help you
maintain a sense of purpose.
Avoid setting unreasonable goals, such as deciding to work full-time while part-time
would be much better for your health.
Stay as active and involved as you can in work and other activities that interest you.
Understanding Hodgkin Lymphoma
The importance of pain control
Patients may feel pain from the cancer itself or from treatments and
procedures. Cancer pain is very treatable and there is no reason for
a patient to tough it out without help. Patients should tell their doctors
and nurses if they have any pain because doctors and nurses can
offer advice regarding medications and other ways to relieve the pain.
Different types of pain are best controlled by different types of pain
relievers:
n
n
n
n
n
Moderate to severe pain is best controlled with opioids such as
morphine, hydromorphone, oxycodone, and hydrocodone.
Part 3
n
Mild to moderate pain can be treated with acetaminophen and
nonsteroidal anti-inflammatory drugs (NSAIDs) such as aspirin and
ibuprofen.
Tingling and burning pain can be controlled with anti-depressants
and anti-epileptics.
Pain caused by swelling can be controlled by steroids such as
prednisone and dexamethasone.
Non-drug pain treatments include: biofeedback, breathing and
relaxation exercises, distraction, heat or cold packs (avoid these
following radiation therapy), hypnosis, imagery, massage, pressure
and vibration, and transcutaneous electrical nerve stimulation
(TENS).
If the pain is not relieved by medicines and non-drug pain
management, the following treatments can also be used to relieve
the source of the pain: radiation therapy, nerve blocks/implanted
pump, neurosurgery, and surgery.
Side Effects and Living With Cancer
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Managing Pain
n
Tell your doctor or nurse about your pain. Be specific when you
describe it.
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– Where do you feel the pain?
– What type of pain is it (sharp, dull, throbbing)?
– Does it come and go, or is it steady?
– How strong is it? How long does it last?
– Does anything make the pain feel better or worse?
– Which drugs have you taken for the pain? Do they help? If so,
for how long?
n
n
n
n
n
Take your pain medication on a regular schedule even if the
pain seems to be better. Do not skip doses.
Tell your family and friends about your pain so that they can
help you and so that they can understand why you may be
acting differently.
Try deep breathing, yoga, or other ways to relax.
Ask to meet with a pain specialist or palliative care specialist to
help you find better ways to control your pain.
Tell your doctor or nurse of any changes in your pain.
Maintain a healthy lifestyle
Regular physical activity helps keep the cardiovascular system strong
and body muscles flexible. Exercise can also help alleviate breathing
problems, constipation, and mild depression. It also helps reduce
stress and fatigue. Patients should talk to their doctor before starting
an exercise program.
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Several types of exercise are particularly helpful:
n
n
n
n
General physical activity, such as swimming, dancing, household
chores, and yard work.
Aerobic activity to improve cardiovascular fitness, such as walking,
jogging, and bicycling.
Resistance training to strengthen muscles, protect joints, and help
remedy osteoporosis by building bone mass.
Flexibility exercises such as stretching and yoga, to improve range
of motion, balance, and stability.
The importance of follow-up care
At the first visit following the completion of treatment, patients should
talk to their doctor about their follow-up schedule. This will be different
from patient to patient depending on their disease type, age, and
general health. Patients should adhere to their schedule of follow-up
visits—these are very important for monitoring disease recurrence
and detecting and treating any health problems that might have been
caused by the treatment.
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Part 3
Eating a healthy diet is especially important during treatment for HL
because it will help patients keep up their strength and energy, tolerate
treatment-related side effects, decrease their risk of infection, and
heal and recover more quickly. Patients should aim for a diet high
in fruits and vegetables, protein (poultry, fish, and eggs), and whole
grains. During chemotherapy and after a stem cell transplant, a patient
may temporarily be asked to avoid raw fruits and vegetables that
may increase the risk of infection if you have a low white blood cell
count (“neutropenic diet”). A patient’s healthcare team can help put
together an eating plan that is appropriate. Patients should talk to their
doctor before taking any dietary supplements such as multivitamins
or individual vitamin supplements, as well as any herbal or “natural”
supplements, because they may interfere with treatments or have
unexpected side effects.
During these follow-up visits, the doctor will ask about any medical
changes since the last appointment and give a physical examination;
the doctor may also prescribe imaging, blood, molecular diagnostic, or
other laboratory tests.
Be proactive in healthcare decisions
To stay proactive in healthcare decisions, patients should get the
following information from their medical team:
n
n
Copies of their medical records and a written summary of their
treatment in case they switch oncologists or need to see a primary
care physician for routine medical care.
A list of signs of disease recurrence and late side effects from
treatment.
At the follow-up care appointments, patients should be sure to tell their
doctor about:
n
Any new symptoms.
n
Pain.
n
n
Physical problems that disrupt their daily life such as fatigue,
insomnia, sexual dysfunction, and weight gain or loss.
Any new health problems, such as heart disease, diabetes, and high
blood pressure.
n
Any new medications and vitamins they are taking.
n
Emotional problems such as anxiety and depression.
n
Any other questions or concerns.
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Part 4 — Clinical Trials and
Advances in Treatment
Chapter 9: Overview of Clinical Trials
There are hundreds of HL clinical trials now underway in hospitals,
cancer centers, and doctors’ offices around the country. The
government, pharmaceutical and biotechnology companies,
universities, and doctor groups often sponsor clinical trials. The United
States Food and Drug Administration (FDA) and the institutional
review board (IRB) of participating hospitals or institutions must
approve all clinical trials.
What is a clinical trial?
A clinical trial is a carefully designed research study that involves
people who volunteer to participate.
In the United States, a new drug must pass through a strict approval
process governed by the FDA before it can become a standard
therapy for use in people. The FDA-regulated approval process for
drugs includes preclinical studies (done in laboratories) and clinical
trials (done in hospitals and clinics).
As shown in the following table, there are 4 main types (or phases)
of clinical trials. The first 3 are usually required before a drug is
considered for approval by the FDA. Phase IV studies are performed
after a drug has received FDA approval; these trials are sometimes
called post-marketing studies. Patients may be eligible to take part in
different types of clinical trials depending on their health status, type
and stage of lymphoma, and type of treatment, if any, they previously
received.
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Part 4
The purpose of clinical trials in cancer is to answer questions about
new ways to treat, diagnose, prevent, or manage the disease or the
side effects caused by treatments. Strict rules and oversight procedures
make sure that clinical trials are designed and run in a way that protects
the rights and safety of the people who volunteer to participate.
Phase
Description
Phase I
n
n
n
Phase II
n
n
Phase III
n
n
Phase IV
n
To find a safe dose.
Number of Volunteer Patients
n
To decide on a treatment
regimen.
15-30 people with one or
more different types of
cancer.
To see if the treatment causes
any side effects.
To find out if a new treatment has
an effect against a certain type of
cancer.
n
Usually fewer than 100
people with the same type
of cancer.
To see if the treatment causes
any side effects.
To compare new treatments or
new uses of existing treatments
with current standard treatments.
The main things being compared
usually are how well the
treatment works and what type of
side effects it causes.
To get more information about
the long-term safety and
effectiveness of a new treatment.
n
n
n
From 100 to several
thousand people with the
same type of cancer.
Patients are randomly
assigned to a treatment
group; one group receives
the standard therapy and
the other group receives the
experimental treatment.
Several hundred to several
thousand people with the
same type of cancer.
Why is a placebo sometimes used in phase III trials?
A placebo is an inactive ingredient that is used as a comparator in
some clinical trials. In such trials neither the patients nor the doctors
and nurses treating them will know which patients receive a placebo
versus the experimental treatment.
In cancer clinical trials, patients are never given a placebo in
place of an effective standard therapy. They would be given a
placebo only if there were no standard therapies to compare against.
Placebo-controlled trials are never done in a manner to deny patients
an effective therapy.
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Understanding Hodgkin Lymphoma
Should I participate in a clinical trial?
Clinical trials are not a last resort for patients. Patients with all stages
of HL can often benefit from participation in clinical trials, whether at
the time of initial diagnosis or at relapse. Clinical trials offer patients
therapies that are not otherwise available to all patients. Another
advantage of clinical trials is that the health of enrolled patients is
monitored very carefully. In many cases, patients can benefit more
from participation in clinical trials than from receiving standard
therapies.
Some professional organizations, like the National Comprehensive
Cancer Network (www.nccn.com) actively encourage the participation
of patients with cancer in clinical trials because they provide the best
management for any cancer patient.
What is informed consent?
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The purpose of the study.
The factors used to decide if a patient is allowed to participate in the
study.
The tests, procedures, and visits participants will be expected to
agree to.
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The type of treatment provided in the study.
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The possible risks and benefits.
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The rights of patients to decide whether or not to participate, and to
leave the study at any time.
The research team will answer questions and provide written
information about the trial. If patients agree to participate in a clinical
trial, they will be asked to read and sign an informed consent form
that details all the trial information discussed and describes how their
records will be kept private.
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Informed consent is a process through which people learn all about
the clinical trials they are interested in joining. During this process,
members of the clinical trial research team will explain:
Remember that even after signing the consent form, patients can
leave the study at any time.
Questions to Ask About a Clinical Trial
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What is the purpose of this clinical trial?
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Why are you recommending this clinical trial for me?
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Who is sponsoring this trial (the National Cancer Institute, a
cancer center, a pharmaceutical/biotechnology company)?
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Who has reviewed and approved this clinical trial?
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Does this clinical trial include the use of a placebo (sugar pill)?
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How long will the study last? Where will it take place?
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What are the risks involved?
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What are the possible benefits?
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What are my responsibilities during the clinical trial?
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TIP
NT
PATIE
What kinds of tests, procedures, or treatments will be
performed? How many and how often?
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Will I be in any discomfort or pain?
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Will I be able to see my own doctor during the clinical trial?
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What costs will I be responsible for?
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What happens if my health gets worse during the clinical trial?
What is the cost of participating in a clinical trial?
Clinical trials are very expensive undertakings for the study sponsor.
Patient costs vary depending on the study, who is sponsoring the
trial, what portion of the trial-related expenses the sponsor will cover,
and the patient’s health insurance coverage. Some health insurance
and managed healthcare providers will pay for the basic medical
procedures associated with the trial, such as lab tests, scans, and
hospitalization when required, while others may define clinical trials as
“experimental” or “investigational” and not cover some of the routine
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costs, such as doctor visits, tests, or treatments. Medicare provides
coverage for patient care associated with government-sponsored
clinical trials.
If a patient is taking part in a National Cancer Institute (NCI) trial being
conducted at the National Institutes of Health (NIH) in Bethesda,
Maryland, the NCI will pay for the study drug and the costs related
to the study. A stipend for travel, food, and lodging is also provided.
Some cancer centers provide financial assistance or discounted rates
for room and board and have special research units that will pay for
study-related costs. Some organizations, including the Lymphoma
Research Foundation, provide financial assistance for treatmentrelated expenses.
Patients should ask their doctor what clinical trials may be most
appropriate for them. Here are some additional sources of clinical trial
information:
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Cancer centers in your area.
The Coalition of Cancer Cooperative Groups website at
www.cancertrialshelp.org.
The NIH websites www.cancer.gov and www.clinicaltrials.gov.
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The Lymphoma Helpline at the Lymphoma Research Foundation at
(800) 500-9976.
Part 4 — Clinical Trials and
Advances in Treatment
Chapter 10: Advances in Treatment of Patients With Hodgkin
Lymphoma
Over 90% of patients with early-stage classical HL and 80% of those
with advanced-stage disease are cured. Because of this high cure
rate, minimizing the long-term effects of therapy is a very important
issue and a very active area of investigation.
Studies are being done to find better ways to identify which patients
need aggressive treatment (which carries an increased risk of longterm side effects) and which can be treated with less aggressive
therapy, without jeopardizing their chance of being cured. Researchers
are also developing treatments that will cause fewer and milder
long-term side effects while curing as many patients as possible.
Doctors and scientists are also looking for more effective and lesstoxic combination regimens with existing drugs (some of which are in
use for other cancers) and with new drugs. New types of drugs being
developed include chemotherapy, targeted therapies, and monoclonal
antibodies.
Drugs that are not yet approved for sale by the FDA are said to be
investigational. The most common way for a patient to receive an
investigational drug is through a clinical trial. To find out more about
getting access to investigational drugs, visit the website for the
National Cancer Institute at www.cancer.gov and search for “access to
investigational drugs.”
Chemotherapy
Researchers are trying to develop new chemotherapy drugs, make
improved versions of existing drugs, and find better ways to combine
different doses and sequences of existing drugs. Some drugs being
studied in patients with HL are already being used for patients with
other cancers.
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Monoclonal antibodies and immunoconjugates
Monoclonal antibodies are molecules made in the laboratory that are
designed to recognize and stick to specific molecules on cancer cells.
When a monoclonal antibody attaches itself to a cancer cell, it can stop
or slow down its growth or it can make it easier for the immune system
to recognize it and kill it. Monoclonal antibodies that target HL-specific
molecules are being developed. For example, a phase III study of the
monoclonal antibody R-mabHDI is currently planned for patients with
lymphocyte predominant HL. Other monoclonal antibodies in phases
I and II trials include HeFi-1, apolizumab, and I 131 tositumomab
(Bexxar), a monoclonal antibody bound to a radioactive molecule.
An immunoconjugate is a monoclonal antibody with a drug attached to
it. In 2011, immunoconjugate brentuximab vedotin (Adcetris) became
the first new drug approved for the treatment of HL in almost 35 years.
Other targeted therapies
Many targeted therapies for HL are being studied in laboratory
experiments and in clinical trials. For example, the mammalian target
of rapamycin (mTOR) inhibitors everolimus (Afinitor) and temsirolimus
(Torisel) are currently being tested in phase II clinical trials. The
histone-deacetylase (HDAC) inhibitors vorinostat, ITF2357, PCI-24781,
and MGCD0103 are also being tested in multiple clinical trials.
Remember that today’s science is moving very fast. Please check with
your doctor for additional information and recent updates.
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In addition to monoclonal antibodies that target molecules on cell
surfaces, many drugs are in development that target molecules inside
cancer cells. A better understanding of the biology and genetics of HL
is helping researchers identify specific molecules in lymphoma cells
that may be good targets for new drugs. These specific molecules
usually have important roles in controlling the growth and survival of
lymphoma cells. The drugs that target these molecules are broadly
called targeted therapies. These drugs may kill the lymphoma cells
or slow down or stop their growth. These targeted therapies attack
cancer cells in a more specific way than chemotherapy drugs.
ABOUT THE LYMPHOMA RESEARCH FOUNDATION
The Lymphoma Research Foundation (LRF) is the nation’s largest
non-profit organization devoted to funding innovative research and
providing people with lymphoma and healthcare professionals with
up-to-date information about this type of cancer. LRF’s mission is to
eradicate lymphoma and serve those touched by this disease.
The Foundation remains dedicated to finding a cure for lymphoma
through an aggressively-funded research program and by supporting
the development of improved therapies for people living with
lymphoma. LRF provides a comprehensive series of programs and
services for people with lymphoma, their loved ones, and caregivers.
How the Lymphoma Research Foundation Serves the
Lymphoma Community
Research
LRF remains dedicated to finding a cure for lymphoma through an
aggressively-funded research program. LRF supports innovative
research through Clinical Investigator Career Development Awards,
Fellowships, and several disease-specific research initiatives. These
focused initiatives include: Chronic Lymphocytic Leukemia/Small
Lymphocytic Lymphoma Initiative; Diffuse Large B-Cell Lymphoma
Initiative; Follicular Lymphoma Initiative; and Mantle Cell Lymphoma
Initiative and Consortium.
The Foundation’s volunteer Scientific Advisory Board (SAB),
comprised of 45 world-renowned lymphoma experts, guides the
Foundation’s research activities, seeking out the most innovative and
promising lymphoma research projects for support.
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Programs and Services
LRF provides a comprehensive series of programs and services for
the lymphoma community, including:
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Clinical Trials Information Service
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Disease-Specific Publications and Websites
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In-person Workshops and Educational Forums
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Lymphoma Helpline
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Lymphoma Support Network
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Online Resources, Teleconferences, and Webcasts/Podcasts
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Patient Aid Grants
Professional Education
LRF is committed to educating healthcare professionals on the latest
developments in lymphoma diagnosis and treatment. The Foundation
offers a wide range of lymphoma-focused continuing education
activities for nurses, physicians, and social workers, including
workshops, conference symposia, and webcasts.
Public Policy and Advocacy
LRF represents the needs of people with lymphoma by supporting
public policies which seek to increase federal funding for lymphoma
research and ensure access to high quality cancer care. The LRF
Advocacy Program provides volunteer advocates with the resources
necessary to garner attention and support for those public policies
most important to the lymphoma community. There are currently more
than 5,000 LRF advocates in all 50 states and the District of Columbia.
About the Lymphoma Research Foundation
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Chapter Network
Through its nationwide chapter network, LRF meets the needs of the
lymphoma community on the local level. Chapter volunteers reach out
to newly diagnosed patients; raise funds for research; and participate
in advocacy efforts. There are 18 chapters in 16 states.
Contact Information
LRF National Headquarters
LRF Helpline
115 Broadway, Suite 1301
New York, NY 10006
(212) 349-2910
Fax (212) 349-2886
[email protected]
www.lymphoma.org
(800) 500-9976
[email protected]
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