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Mantle Cell
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NCCN Guidelines for Patients®
Version 1.2016
Mantle Cell Lymphoma
Learning that you have cancer can be overwhelming. The goal of this book is
to help you get the best cancer treatment. It explains which cancer tests and
treatments are recommended by experts of mantle cell lymphoma.
The National Comprehensive Cancer Network® (NCCN®) is a not-for-profit
alliance of 27 of the world’s leading cancer centers. Experts from NCCN® have
written treatment guidelines for doctors who treat mantle cell lymphoma. These
treatment guidelines suggest what the best practice is for cancer care. The
information in this patient book is based on the guidelines written for doctors.
This book focuses on the treatment of mantle cell lymphoma. Key points of the
book are summarized in the related NCCN Quick Guide™. NCCN also offers
patient resources on follicular lymphoma, diffuse large B-cell lymphoma, chronic
lymphocytic leukemia, and other cancer types. Visit NCCN.org/patients for the
full library of patient books, summaries, and other resources.
NCCN Guidelines for Patients®
Mantle Cell Lymphoma, Version 1.2016
1
Credits
NCCN aims to improve the care given to patients with cancer. NCCN staff work with experts to create helpful programs and resources for many
stakeholders. Stakeholders include health providers, patients, businesses, and others. One resource is the series of books for patients called
the NCCN Guidelines for Patients®. Each book presents the best practice for a type of cancer. The patient books are based on clinical practice
guidelines written for cancer doctors. These guidelines are called the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®). Clinical
practice guidelines list the best health care options for groups of patients. Many doctors use them to help plan cancer treatment for their patients.
Panels of experts create the NCCN Guidelines®. Most of the experts are from NCCN Member Institutions. Panelists may include surgeons,
radiation oncologists, medical oncologists, and patient advocates. Recommendations in the NCCN Guidelines are based on clinical trials and the
experience of the panelists. The NCCN Guidelines are updated at least once a year. When funded, the patient books are updated to reflect the
most recent version of the NCCN Guidelines for doctors. For more information about the NCCN Guidelines, visit NCCN.org/clinical.asp.
NCCN staff involved in making the guidelines for patients and doctors include:
NCCN Guidelines for Patients
NCCN Guidelines
NCCN Marketing
Dorothy A. Shead, MS
Director
Patient and Clinical Information Operations
Mary Dwyer, MS
Senior Manager, Guidelines
Susan Kidney
Graphic Design Specialist
Laura J. Hanisch, PsyD
Medical Writer/Patient Information Specialist
Lacey Marlow
Associate Medical Writer
Hema Sundar, PhD
Oncology Scientist/Senior Medical Writer
Rachael Clarke
Guidelines Data and Layout Coordinator
Endorsed by
THE Leukemia & lymphoma society (LLS)
LLS is dedicated to developing better outcomes for blood cancer patients through research, education and patient services
and is happy to have this comprehensive resource available to patients with mantle cell lymphoma.
www.LLS.org/informationspecialists
Supported by NCCN Foundation®
The NCCN Foundation supports the mission of the National Comprehensive Cancer Network® (NCCN®)
to improve the care of patients with cancer. One of its aims is to raise funds to create a library of books for
patients. Learn more about the NCCN Foundation at NCCN.org/foundation.
© 2016 National Comprehensive Cancer Network, Inc. All rights reserved. The NCCN Guidelines for Patients® and illustrations
herein may not be reproduced in any form for any purpose without the express written permission of NCCN.
National Comprehensive Cancer Network (NCCN)
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Fort Washington, PA 19034
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NCCN Guidelines for Patients®
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Contents
Mantle Cell Lymphoma
How to use this book
61
Glossary:
5 Part 1
62
Dictionary
66Acronyms
Explains how and where this cancer starts.
69
NCCN Panel Members
15 Part 2
70
NCCN Member Institutions
Describes how doctors plan your
72
Index
4
Mantle cell lymphoma basics
Treatment planning
treatment.
25 Part 3
Overview of cancer
treatments
Describes the treatments used to cure or
control mantle cell lymphoma.
37 Part 4
Treatment guide
Presents treatment options based on the
cancer stage.
51 Part 5
Making treatment decisions
Offers tips for choosing the best treatment.
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How to use this book
Who should read this book?
The recommendations in this book are based on
science and the experience of NCCN experts.
However, these recommendations may not be
right for you. Your doctors may suggest other
tests and treatments based on your health
and other factors. If other recommendations
are given, feel free to ask your treatment team
questions.
Mantle cell lymphoma is the focus of this book.
Information on diagnosis, treatment planning,
and treatment options are included. People with
mantle cell lymphoma and those who support
them—caregivers, family, and friends—may find
this book helpful. It may help you discuss and
decide with doctors what care is best.
Making sense of medical
terms
Where should I start
reading?
In this book, many medical words are included.
These are words that you will likely hear from
your treatment team. Most of these words may
be new to you, and it may be a lot to learn.
Starting with Part 1 may be helpful. It explains
what mantle cell lymphoma is and how it is
diagnosed. Parts 2 through 5 address issues
related to treatment. Part 2 lists which health
tests and other types of care are needed before
treatment. Part 3 briefly describes all the types
of treatments so you can understand your
options that are listed in Part 4. Tips for making
treatment decisions are presented in Part 5.
Don’t be discouraged as you read. Keep reading
and review the information. Don’t be shy to ask
your treatment team to explain a word or phrase
that you do not understand.
Words that you may not know are defined in the
text or in the Dictionary. Words in the Dictionary
are underlined when first used on a page.
Does the whole book
apply to me?
Acronyms are also defined when first used
and in the Glossary. Acronyms are short words
formed from the first letters of several words.
One example is DNA for deoxyribonucleic acid.
This book includes information for many
situations. Your treatment team can help. They
can point out what information applies to you.
They can also give you more information. As
you read through this book, you may find it
helpful to make a list of questions to ask your
doctors.
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1
Mantle cell lymphoma basics
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1 Mantle cell
lymphoma basics
6 What is the lymphatic system?
8 What is mantle cell lymphoma?
10Do I have mantle cell lymphoma?
14Review
What is the lymphatic system?
You’ve learned that you have or
may have mantle cell lymphoma.
It’s common to feel shocked and
confused. Part 1 reviews some
basics that may help you learn about
this lymphoma and start to cope.
These basics may also help you start
planning for treatment.
The lymphatic system is one of 13 systems of the
human body. It transports fluids to the bloodstream
and fights germs. As such, it supports your bloodflowing (cardiovascular) and disease-fighting
(immune) systems.
Cells are the building blocks of tissue in the body. The
spaces between cells are filled with fluid. This fluid
is called interstitial or tissue fluid. Most tissue fluid
comes from parts of blood plasma that have passed
out of blood vessels. Cells also release waste and
other products into tissue fluid.
When tissue fluid increases, it drains into vessels.
Almost all of tissue fluid drains back into blood
vessels. The rest of it drains into lymph vessels.
Once inside of lymph vessels, tissue fluid is called
lymph. Lymph travels in lymph vessels back to the
bloodstream.
The lymphatic system also collects fat and some
vitamins from your gut. After you eat, your stomach
turns food into a liquid. Then, the liquid drains into
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Mantle cell lymphoma basics
What is the lymphatic system?
your small intestine. Within your small intestine, fat
and some vitamins are absorbed into lymph vessels.
This fatty lymph, called chyle, travels in lymph vessels
to the bloodstream.
Lymphocytes help fight germs. The three types of
lymphocytes are NK (natural killer) cells, B-cells, and
T-cells. Lymphocytes are made in bone marrow then
are moved by blood to the lymphatic system.
As lymph travels, it will pass through and be filtered
by lymph nodes. Lymph nodes are organized masses
of lymphoid tissue. There are hundreds of lymph
nodes throughout your body. See Figure 1.1. High
numbers of lymph nodes exist in the middle of your
chest, neck, armpit, groin, pelvis, and along your gut.
Other parts of your body that have many lymphocytes
are included in the lymphatic system. In children,
the thymus stores T-cells until they are able to fight
germs. Germs in blood are filtered and destroyed
by lymphocytes within your spleen. Your tonsils kill
germs in lymph that enter through your mouth and
nose. There are also small clumps of lymphatic tissue
in your gut, thyroid, breasts, lungs, eyes, and skin.
Lymph nodes and other lymphoid tissue are
defined by high numbers of white blood cells
called lymphocytes. Lymph also has lymphocytes.
Figure 1.1
Lymphatic system
The lymphatic system kills germs in the body and collects and transports lymph to the bloodstream.
Illustration Copyright © 2016 Nucleus Medical Media, All rights reserved. www.nucleusinc.com
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Mantle cell lymphoma basics
What is mantle cell lymphoma?
What is mantle cell lymphoma?
from B-cells that have passed through germinal
centers.
Cancer is a disease of cells. Lymphomas are cancers
that start in lymphocytes within the lymphatic system.
There are two main types of lymphomas. Hodgkin
lymphoma is defined by the presence of ReedSternberg or related cells. Non-Hodgkin’s lymphoma
includes all the other types of lymphoma.
Inside of cells are coded instructions for building
new cells and controlling how cells behave. These
instructions are called genes. Genes are a part
of DNA (deoxyribonucleic acid), which is grouped
together into bundles called chromosomes. See
Figure 1.2. Abnormal changes (mutations) in genes
cause normal B-cells to become cancer cells.
Researchers are still trying to learn what causes
genes to mutate and cause cancer.
Most non-Hodgkin’s lymphomas—85 out of every
100—are B-cell lymphomas. About 10 out of 100 are
T-cell lymphomas. A few have unknown cell origin.
It is now known that most Hodgkin lymphomas are
also from B-cells. Thus, Hodgkin and non-Hodgkin’s
lymphomas are more related than first thought.
Cancer cells don’t behave like normal cells. First, the
mutations cause cancer cells to grow more quickly
and live longer than normal cells. Normal cells grow
and then divide to form new cells when needed.
They also die when old or damaged as shown in
Figure 1.3. In contrast, cancer cells make new cells
that aren’t needed and don’t die quickly when old or
damaged. Over time, the lymphoma cells may build
up in tissues and may travel in blood or lymph to
other sites. Without treatment, the cancer may cause
organs not to work.
Mantle cell lymphoma is a type of non-Hodgkin’s
lymphoma. It is a cancer of B-cells. There are many
types of B-cells and thus, many B-cell cancers.
B-cells differ from one another based on the cell’s
stage of development. As B-cells “mature” they
change in their ability to make antibodies.
Antibodies are Y-shaped proteins that are made in
response to the presence of antigens. Some antigens
enter your body from outside. Such antigens include
viruses, bacteria, chemicals, and pollen. Some
antigens are formed inside your body like those found
on tissue cells. Antibodies attach to antigens, which
triggers a response from your immune system.
Germinal centers are short-lived structures within
lymph nodes that are formed in response to an
outside antigen. Within germinal centers, B-cells
undergo changes to prepare them for making
antibodies. Most mantle cell lymphomas start from
B-cells that have not passed through a germinal
center. These “naïve” B-cells can be found in mantle
zones that surround germinal centers. Of interest,
a subset of mantle cell lymphomas appears to start
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Mantle cell lymphoma basics
What is mantle cell lymphoma?
Figure 1.2
Genetic material in cells
Most human cells contain the “blueprint of life”—the plan by which our bodies are made
and work. The plan is found inside of chromosomes, which are long strands of DNA that are
tightly wrapped around proteins. Genes are small pieces of DNA that contain instructions for
building new cells and controlling how cells behave. Humans have about 24,000 genes.
Illustration Copyright © 2016 Nucleus Medical Media, All rights reserved. www.nucleusinc.com
Figure 1.3
Normal cell growth vs. cancer cell growth
Normal cells increase in number when they are needed and die when old or damaged.
In contrast, cancer cells quickly make new cells and live longer because of abnormal
changes in genes.
Illustration Copyright © 2016 Nucleus Medical Media, All rights reserved. www.nucleusinc.com
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Mantle cell lymphoma basicsDo I have mantle cell lymphoma?
Do I have mantle cell
lymphoma?
diagnosing blood cancers. The hematopathologist will
first examine the samples using a microscope.
One of the most common symptoms of mantle cell
lymphoma is a swelling of one or more lymph nodes.
Swollen nodes may occur in your neck, armpit, or
groin. However, mantle cell lymphoma is often found
elsewhere in your body. Other common sites are the
GI (gastrointestinal) tract and bone marrow. The GI
tract is a group of organs in which food is broken
down, absorbed, and disposed of. Symptoms of GI
tumors include pain in your gut, diarrhea, and blood in
your stool. When your doctor suspects cancer, testing
is needed. The tests that are needed to confirm
(diagnose) mantle cell lymphoma are described next.
The hematopathologist will assess the rate of cancer
growth by noting how the cells look. Compared to
other lymphomas, most mantle cell lymphomas do not
grow slowly but do not grow very fast either. Doctors
describe the growth as “moderately aggressive.”
However, there are some mantle cell lymphomas that
grow slowly and others quite fast.
Biopsy
Protein tests
FNA (fine-needle aspiration) removes very small
samples with a needle. It should not be used alone
to diagnose mantle cell lymphoma. You may have
cancer even if no cancer is found in the samples. FNA
with a core biopsy may work well enough.
The IHC panel should test for BCL2, BCL6, CD3,
CD5, CD10, CD20, CD21, CD23, cyclin D1, and
Ki-67. Mantle cell lymphoma cells often have CD5,
CD20, CD43, and high levels of cyclin D1. Rare
cases may have normal cyclin D1 levels. CD10
and CD23 are found on most but not all mantle cell
lymphoma cells.
The results of the review and the tests described next
will be recorded in a pathology report. It’s a good idea
to get a copy of your pathology report. It’s used to
plan treatment.
Tissue or fluid must be removed from your body and
be tested to diagnose cancer. A biopsy removes
samples of fluid or tissue. To diagnose mantle cell
lymphoma, an incisional or excisional biopsy of the
tumor is advised. An incisional biopsy removes only a
part of the tumor through a cut made into your body.
An excisional biopsy removes the whole tumor and
not much else. The methods used to do either biopsy
depend on where the tumor is in your body.
For diagnosis, the hematopathologist needs to study
the proteins in the cells’ surface (membrane). This is
called immunophenotyping. Mantle cell lymphoma
has a common pattern or “signature” of proteins. See
Figure 1.4. An IHC (immunohistochemistry) panel
is a test for such proteins. It involves applying a
chemical marker to cells then looking at them with a
microscope.
Hematopathology review
The biopsy samples will be sent to a pathologist.
A pathologist is a doctor who’s an expert in testing
cells to find disease. To diagnose mantle cell
lymphoma, the pathologist should be a specialist
in hematopathology. Hematopathologists spend
all of their time looking at blood and bone marrow
and lymph nodes, so they become very good at
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Sometimes it is helpful to include LEF1 and SOX11
in the IHC panel. If LEF1 is found, the cancer is likely
chronic lymphocytic leukemia and not mantle cell
lymphoma. SOX11 is found in almost every case
of mantle cell lymphoma. Thus, SOX11 is helpful
for diagnosis when the cells have normal levels of
cyclin D1. On the other hand, a low or absent SOX11
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1
Mantle cell lymphoma basicsDo I have mantle cell lymphoma?
Figure 1.4 CD20 protein
Mantle cell lymphoma cells have a common pattern of proteins in their membrane. This
pattern includes the presence of CD5, CD20, CD43, and high levels of cyclin D1. CD10
and CD23 are found on some but not all cells. Immunophenotyping is the process of
identifying the proteins in cells’ membranes.
Derivative work of Rituximab Binding to CD20 on a B Cell Surface by NIAID available at commons.wikimedia.org/wiki/
File:Rituxima_Binding_to_CD20_on_a_B_Cell_Surface_(6830897205).jpg under a Creative Commons Attribution 2.0
Generic license.
level is a good marker of slow-growing mantle cell
lymphoma.
Flow cytometry is a newer method that can also be
used to assess the surface proteins on lymphoma
cells. This method involves first adding a marker—a
light-sensitive dye—to cells. Then, your blood will
be passed through a flow cytometry machine. The
machine measures surface proteins on thousands of
cells.
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Flow cytometry may be done in addition to an IHC
panel. If done, it should test for CD5, CD10, CD19,
CD20, CD23, and kappa and lambda light chain
proteins. Light chain proteins are part of antibodies. It
may be helpful to test for CD200 if the type of cancer
is unclear. Mantle cell lymphoma cells do not have
CD200 whereas chronic lymphocytic leukemia cells
do.
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Mantle cell lymphoma basicsDo I have mantle cell lymphoma?
Genetic tests
from B-cells that have passed through germinal
centers. They often have low or absent SOX11 levels.
It may be useful to test for IGHV mutations since they
are a sign of slow-growing mantle cell lymphoma.
DNA sequencing is a lab test of blood or marrow
that is used to look for mutations in genes. This test
reveals the order of the chemicals that make up DNA.
Besides a protein signature, mantle cell lymphomas
often have a translocation. A translocation is a
switching of parts between two chromosomes. The
translocation in mantle cell lymphoma occurs between
chromosomes 11 and 14—referred to as t(11;14).
See Figure 1.5. This translocation often involves
the switching of the CCND1 gene on chromosome
11 with the IgH locus on chromosome 14. The result
is too much cyclin D1. If cyclin D1 is normal on the
IHC panel, it may be useful to test for t(11;14). Tests
that detect translocations are a karyotype or FISH
(fluorescence in situ hybridization).
Other genetic tests may be useful if the type of
cancer is unclear. A karyotype or FISH to test for
translocation of chromosomes 14 and 18 may be
done. This translocation is often found in follicular
lymphoma and sometimes diffuse large B-cell
lymphoma. If present, the cancer is unlikely mantle
cell lymphoma. Likewise, the cancer is likely to be
chronic lymphocytic leukemia if a FISH panel shows
defects in chromosomes 11, 12, 13, or 17.
A subgroup of mantle cell lymphomas has mutations
in the IGHV (immunoglobulin heavy-chain variable)
region genes. These cancers appear to have started
Figure 1.5 Translocation
A translocation is a switching of parts between chromosomes. In mantle cell lymphoma
cells, a translocation between chromosomes 11 and 14 is often present. The result is too
much cyclin D1 in the surface of cancer cells.
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Mantle cell lymphoma basicsDo I have mantle cell lymphoma?
My notes
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1
Mantle cell lymphoma basicsReview
Review
• The lymphatic system consists of lymph and
a network of vessels and organs. It helps kill
germs in the body and transports fluids to the
bloodstream.
• Incisional or excisional biopsy is needed to
diagnose mantle cell lymphoma.
• Most mantle cell lymphomas grow and spread
moderately fast. However, some do grow
slowly and others very fast.
• Lymphomas are cancers that start in
lymphocytes within the lymphatic system.
Mantle cell lymphoma is a cancer of B-cells.
Most of these B-cells are from mantle zones
within lymphatic organs.
NCCN Guidelines for Patients®
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• The biopsy tissue should be tested by a
hematopathologist. The hematopathologist will
perform a number of tests that assess for cell
type, surface proteins, and maybe genetics.
14
2
Treatment planning
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2 Treatment planning
16Medical history
17Physical exam
18
Blood tests
19Imaging tests
20Scopes
21
Bone and marrow test
22
Spinal fluid test
23
Heart tests
23
Fertility and pregnancy
24Review
Medical history
Doctors plan treatment with many
sources of information. One of these
sources is tests of your health and the
cancer. Part 2 describes who should
receive which tests before treatment.
Some of these tests are repeated
during and after treatment. Besides
tests, Part 2 describes other types
of care that are important to receive
before cancer treatment.
Your medical history includes any health events and
medicines you’ve taken in your life. You will be asked
about illnesses, injuries, health conditions, and more.
Some health problems run in families. Thus, your
doctor may also ask about the health of your blood
relatives.
Some signs and symptoms of mantle cell lymphoma
are enlarged lymph nodes, tiredness, and a feeling
of fullness in your belly. This cancer may also cause
“B symptoms.” It’s important that your doctor knows
if you have them. These symptoms include fevers,
chills, night sweats, and weight loss without dieting.
A medical history is one of the tests needed for
treatment planning. See Chart 2.1 for a complete
list of care that is recommended prior to treatment.
Some types of care are for most people with mantle
cell lymphoma while other types may be useful for a
subset of people.
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Treatment planningPhysical exam
Physical exam
Doctors sometimes give a physical exam along with
taking a medical history. A physical exam is a study of
your body for signs of disease. During this exam, your
doctor will listen to your lungs, heart, and gut. Parts
of your body will likely be felt to see if organs are of
normal size, are soft or hard, or cause pain when
touched.
For mantle cell lymphoma, there are certain parts
of your body that should be checked. Mantle cell
lymphoma is often found in lymph nodes, so areas
with lots of lymph nodes should be examined. High
numbers of lymph nodes exist in the middle of your
chest, neck, throat, armpit, groin, pelvis, and along
your gut. The size of your spleen and liver should also
be assessed.
Results of your medical history and physical exam
will be used to rate your performance status.
Performance status is your ability to do daily
activities. It is used by doctors to assess if you can
undergo certain treatments. The ECOG (Eastern
Cooperative Oncology Group) Performance Scale is
a common scoring scale for performance status. This
scale consists of scores from 0 to 4. Lower scores
mean you can do more activities.
Chart 2.1 Care before treatment
Must haves
Sometimes useful
• Medical history
• Beta-2-microglobulin
• Physical exam
• Uric acid
• Complete blood count with differential
• Neck CT
• Comprehensive metabolic panel
• Endoscopy or colonoscopy
• Lactate dehydrogenase
• Lumbar puncture
• Hepatitis B testing
• Fertility support
• Diagnostic CT or PET/CT
• Bone marrow biopsy ± aspiration
• Echocardiogram or MUGA if you may
receive certain types of chemotherapy
• Pregnancy test if you can have babies
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Treatment planning
Blood tests
Blood tests
Hepatitis B testing Hepatitis B can be an important factor in the
treatment of mantle cell lymphoma. Hepatitis B can
become active again due to the cancer or some of its
treatments. Thus, tell your treatment team if you’ve
ever been infected with hepatitis. If you’ve never been
tested, ask your treatment team if you should. You
may need to be tested if you will be treated with a
drug called rituximab.
Blood tests are used to learn if cancer treatment
might be needed now. They are also used to find
unknown diseases including those related to mantle
cell lymphoma. It’s important to treat the cancer as
well as other health problems. Blood tests for mantle
cell lymphoma are:
Complete blood count with differential
A CBC (complete blood count) measures the number
of blood cells in a blood sample. It includes numbers
of white blood cells, red blood cells, and platelets.
Your blood counts may be low or high because of
cancer or another health problem. It is an essential
test that helps your doctor learn about your overall
health.
Beta-2 microglobulin
There are several types of white blood cells. A
differential counts the number of each type. It also
checks if the counts are in balance with each other.
Your doctor can determine the cause of an abnormal
white blood count from this test.
Uric acid
Beta-2 microglobulin is a small protein made by
many types of cells, including lymphoma cells. It is
measured with a blood chemistry test. If related to
cancer, high levels may be a sign that the cancer is
widespread. This test may be useful to gauge the
extent of the cancer.
Some people with mantle cell lymphoma are at
risk for tumor lysis syndrome (TLS, for short). This
syndrome can be life threatening. It occurs when the
waste released by dead cells is not quickly cleared
out of your body. This results in kidney damage and
severe blood electrolyte disturbances.
Comprehensive metabolic panel
Chemicals in your blood come from your liver, bone,
and other organs. A comprehensive metabolic panel
often includes tests for up to 14 chemicals. The tests
show if the level of chemicals is too low or high.
Abnormal levels can be caused by cancer or other
health problems.
Tumor lysis syndrome can occur among people with
mantle cell lymphoma who are undergoing strong
cancer treatments. The cancer treatment kills many
cancer cells and results in too much tumor cell waste
within in a short period of time.
Lactate dehydrogenase
Your doctors may want to know your uric acid level
before starting treatment. You may be given certain
medications that can help prevent tumor lysis
syndrome. Also, drinking plenty of water throughout
chemotherapy can help. Ask your treatment team for
more information.
Lactate dehydrogenase is a protein that is in most
cells. It gets into your blood when a cell is damaged.
Thus, a high level of lactate dehydrogenase is a sign
of cell damage. High levels can be caused by cancer
or other health problems. If related to cancer, high
levels may be a sign that the cancer is widespread.
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2
Treatment planningImaging tests
Imaging tests
Figure 2.1 Computed tomography machine
Imaging tests make pictures (images) of the inside of
your body. They can show which sites have cancer.
This information helps your doctors stage the cancer.
More information on cancer staging is in Part 4.
A CT machine is large and has a tunnel in the
middle. During the test, you will lie on a table that
moves slowly through the tunnel.
Your treatment team will tell you how to prepare for
the test. You may need to stop taking some medicines
and stop eating and drinking for a few hours before the
scan. Tell your doctors if you get nervous when in small
spaces. You may be given a sedative to help you relax.
Imaging machines are large. You will likely be lying
down during testing. At least part of your body will
be in the machine. Figure 2.1 shows one type of an
imaging machine.
After the test, you will likely be able to resume your
activities right away. If you took a sedative, you will
have a waiting period. You may not learn of the
results for a few days since a radiologist needs to see
the pictures. A radiologist is a doctor who’s an expert
in reading the images.
Diagnostic CT
A CT (computed tomography) of your chest, belly
area, and between your hip bones is needed. A CT
of your neck is sometimes useful to learn if cancer is
present. CT takes many pictures of a body part from
different angles using x-rays. A computer combines
the x-rays to make detailed pictures.
A contrast dye is used for diagnostic CT. It makes the
pictures clearer. The dye will be injected into a vein
in your hand or arm. You will also be given a liquid
contrast to drink.
The contrast may cause you to feel flushed or get
hives. Rarely, serious allergic reactions occur. Tell
your doctor and the technicians if you have had
problems with contrast in the past.
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PET/CT
Another imaging test combines CT with PET (positron
emission tomography). PET/CT may be given in
addition to or instead of CT. It can show the presence
of cancer when other tests do not.
For PET, a sugar radiotracer will be injected into
your body. The radiotracer is detected with a special
camera. Cancer cells appear brighter than normal
cells because they use sugar more quickly. PET/CT
may be done with one or two machines depending on
the cancer center.
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Treatment planningScopes
Scopes
Figure 2.2 Colonoscopy
It is common for mantle cell lymphoma to spread
to the GI tract. Endoscopy and colonoscopy are
procedures during which a “scope” is inserted into the
GI tract to remove tissue samples for cancer testing.
Testing for cancer in your GI tract may not be needed
if it would not affect your treatment options. NCCN
experts do advise GI testing if your doctor thinks that
the cancer isn’t widespread in order to confirm local
disease. A second scope will be done to test for how
well treatment worked for local disease.
Tissue samples from your GI tract may be removed
to test for cancer. The tissue samples from your
colon may be removed during a procedure called
a colonoscopy as shown below. An endoscopy is
a procedure that removes tissue samples from the
upper GI tract, which includes your esophagus and
stomach.
Endoscopy An endoscopy allows your doctor to see the insides
of your esophagus and stomach. It is done with an
endoscope. An endoscope has a thin, long tube
that will be guided into your body, often through the
mouth. At the end of the tube is a very small light,
camera lens, and cutting tool. At the other end of
the endoscope is an eyepiece that your doctor looks
through to see the images shown by the camera.
You will likely be sedated during the exam, but
sometimes general anesthesia is used. Air may be
pumped into your esophagus and stomach for better
viewing. After the exam, your throat may feel sore and
you may feel bloated from the air.
Illustration Copyright © 2016 Nucleus Medical Media, All rights
reserved. www.nucleusinc.com
Colonoscopy
A colonoscopy allows your doctor to see inside your
colon. It is done with a colonoscope, which looks and
works much like an endoscope. You will be asked to
wear a hospital gown and lie on your side during the
exam as shown in Figure 2.2.
picture from the colonoscope will be viewed by your
doctor on a screen.
To prepare for this test, your doctor may place you on
a liquid diet for 1 to 3 days. You may also be given a
laxative or an enema to clean out your intestine the
night before the test. Right before the test, you may
be given a sedative. The day after the test, you are
likely to feel normal. If you have severe pain, bloody
stools, or weakness, contact your doctor.
The colonoscope will be inserted into your anus and
gently guided through your intestine. Air may be
pumped into your intestine to make it bigger for better
viewing. You may be asked to shift a little during the
test to help your doctor guide the colonoscope. The
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Mantle Cell Lymphoma, Version 1.2016
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Treatment planning
Bone and marrow test
Bone and marrow test
Figure 2.3 Bone marrow biopsy
Mantle cell lymphoma is often but not always found
in bone marrow at diagnosis. Thus, a bone marrow
biopsy with or without aspiration is needed to confirm
there is cancer. A bone marrow biopsy removes
a sample of bone and soft bone marrow. A bone
marrow aspiration removes a small amount of liquid
bone marrow.
Doctors use a bone marrow biopsy to remove a
sample of bone and marrow for testing.
Often, these tests are done at the same time on the
back of hip bone. You may receive a light sedative
before the test. You will likely lie on your side as
shown in Figure 2.3. Some people lie on their belly.
Your doctor will clean your skin then give local
anesthesia to numb the site. Once numb, a hollow
needle will be inserted into your skin and then pushed
into the bone to remove the liquid bone marrow with a
syringe. Then, a wider needle will be inserted into the
bone and rotated to remove bone and soft marrow.
These biopsies may cause bone pain and can bruise
your skin for a few days. The samples will be sent to
a lab for testing.
Illustration Copyright © 2016 Nucleus Medical Media, All rights
reserved. www.nucleusinc.com
NCCN Guidelines for Patients®
Mantle Cell Lymphoma, Version 1.2016
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Treatment planning
Spinal fluid tests
Spinal fluid tests
Figure 2.4 Lumbar puncture
Mantle cell lymphoma can spread into the fluid
around your brain and spinal cord. This fluid is called
cerebrospinal fluid or spinal fluid. To confirm that
cancer is in spinal fluid, a sample must be removed
and tested.
A lumbar puncture is used to remove a sample of
spinal fluid so it can be tested for cancer. A lumbar
puncture may also be used to inject cancer drugs
into spinal fluid.
A lumbar puncture is a procedure that removes spinal
fluid. It is also called a spinal tap. A lumbar puncture
is advised if you have the blastoid type of mantle cell
lymphoma or have symptoms of cancer in your CNS
(central nervous system). Such symptoms include a
loss of balance, loss of sensation, muscle weakness,
seizures, headaches, and changes in vision.
During a spinal tap, you will be lying down or sitting
on an exam table. If lying down, your knees must
be tucked up near your chest. If sitting, you must
lean slightly forward and down toward your knees as
shown in Figure 2.4. The lower part of your back over
your spine will be numbed with a local anesthetic.
Next, a thin needle will be inserted between the
bones of your spine and into the space around your
spinal cord. You may feel some pressure during the
procedure. The fluid sample will then be sent to a lab
for testing.
NCCN Guidelines for Patients®
Mantle Cell Lymphoma, Version 1.2016
Illustration Copyright © 2016 Nucleus Medical Media, All rights
reserved. www.nucleusinc.com
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Treatment planning
Heart tests | Fertility and pregnancy
Heart tests
Fertility and pregnancy
Some cancer treatments can damage your heart.
Thus, your doctor may test how well your heart works
to plan treatment. You may receive one of the two
tests described next. If your heart isn’t working well,
you may receive other treatment.
Some cancer treatments can limit your ability to have
a baby. If you want the choice of having babies after
treatment or are unsure, tell your doctors. It may also
help to talk with a fertility specialist before you begin
cancer treatment. A fertility specialist is an expert in
helping people have babies. The fertility specialist
can discuss with you how to have a baby after
treatment. Some methods of fertility preservation are
discussed next. If you are a woman of childbearing
age, important information on pregnancy is also
addressed.
Sperm banking
An echocardiogram is an imaging test of your heart.
It uses sound waves (ultrasound) to make pictures.
During this test, you will be lying down. Small patches
will be placed on your chest to track your heartbeat.
Next, a probe with gel on its tip will be slid across part
of your bare chest. A picture of your beating heart
will be seen at once on a screen. The pictures will be
recorded for future viewing.
A MUGA (multi-gated acquisition) scan measures
how well your heart is pumping blood. For this test,
patches will be placed on your chest to track your
heartbeat. Also, a radiotracer will be injected into
your vein. Pictures of your heart will be taken with a
special camera that can detect the radiation released
by the tracer.
NCCN Guidelines for Patients®
Mantle Cell Lymphoma, Version 1.2016
Men who want to father children after cancer
treatment can use sperm banking. Sperm banking
stores semen for later use. This is done by freezing
semen with sperm in liquid nitrogen. Talk to your
treatment team about the costs of and how well
sperm banking works.
Egg freezing and more
Like sperm banking, a woman’s eggs can be
removed, frozen, and stored for later use. Your frozen
eggs can be fertilized with sperm beforehand. Also,
a part of your ovary that contains eggs can be frozen
and stored.
Pregnancy test
Some cancer treatments can harm an unborn baby.
Get a pregnancy test before treatment if you may be
pregnant now. Your treatment options will depend
on the results. During treatment, take steps to avoid
getting pregnant. Your doctors can tell you which birth
control methods are best to use while on treatment.
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Treatment planningReview
Review
• Tell your doctor if you have recently had
fevers, night sweats, and weight loss without
dieting. These can be symptoms of mantle cell
lymphoma.
• A bone marrow biopsy removes a piece of
bone and marrow to test for cancer cells. This
test is needed to plan treatment. In addition
to the biopsy, an aspiration to remove liquid
marrow may be done, too.
• Your doctor will examine your body for signs
of disease. He or she will check if your lymph
nodes, liver, or spleen are large. Your doctor
will also rate your ability to do everyday
activities.
• A lumbar puncture may be needed to confirm
if the cancer has spread into your spinal fluid.
• You may undergo heart tests to see if you
are healthy enough to have certain cancer
treatments.
• Blood tests can be done to assess for signs
of widespread cancer and for other health
conditions.
• Talk to a fertility specialist to learn about ways
to have babies after cancer treatment. If you
may be pregnant now, get a pregnancy test
since some cancer treatments can harm
unborn babies.
• Imaging tests allow your doctors to see inside
your body without cutting into it. Diagnostic CT
or PET/CT of your torso is needed. Neck CT
may be useful.
• Colonoscopy or endoscopy may be done to
collect tissue samples from your GI tract in
order to confirm local disease and treatment
response.
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3
Overview of
cancer treatments
NCCN Guidelines for Patients®
Mantle Cell Lymphoma, Version 1.2016
25
3 Overview of cancer
treatments
26Chemotherapy
28Steroids
29
Targeted therapy
30Immunomodulators
30Stem cell transplant
32Radiation therapy
34
Clinical trials
36Review
Chemotherapy
In Part 3, the main treatment types
for mantle cell lymphoma are briefly
described. Knowing what a treatment
is will help you understand your
treatment options listed in Part 4.
There is more than one treatment
for mantle cell lymphoma. Not every
person will receive every treatment
described in this chapter.
Chemotherapy, or “chemo,” includes drugs that
disrupt the life cycle of cancer cells so they can’t
increase in number. Chemotherapy along with other
cancer drugs is almost always first used to treat
mantle cell lymphoma.
Some chemotherapy drugs kill cancer cells by
damaging their DNA or by disrupting the making of
DNA. Other drugs interfere with cell parts that are
needed for making new cells. Thus, no new cells are
made to replace dying cells.
Many chemotherapy drugs work when cells are in
an active growth phase. During the active growth
phase, cells grow and divide to form a new cell.
Chemotherapy drugs that disrupt the growth phase
work well for cancer cells that are growing and
dividing quickly. Other chemotherapy drugs work
whether cells are in a growth or resting phase.
Chemotherapy can kill both cancer and normal cells.
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Overview of cancer treatments
Chemotherapy
Most chemotherapy drugs for mantle cell lymphoma
are liquids that are slowly injected into a vein. A few
are made as pills or can be injected under the skin.
By any method, the drugs travel in your bloodstream
to treat cancer throughout your body. Doctors use
the term “systemic” when talking about a cancer
treatment for the whole body. Chemotherapy and
other drugs used to treat mantle cell lymphoma are
listed in Chart 3.1.
Chart 3.1 Drug treatment for mantle cell lymphoma
Generic name
Brand name (sold as)
Type of treatment
Bendamustine hydrochloride
Treanda®
Chemotherapy
Bortezomib
Velcade
Chemotherapy
Carboplatin
–
Chemotherapy
Carmustine
–
Chemotherapy
Cisplatin
Platinol®
Cladribine
Leustatin
Cyclophosphamide
–
Cytarabine
Cytosar-U
Dexamethasone
–
Steroid
Doxorubicin hydrochloride
–
Chemotherapy
Etoposide; Etoposide phosphate
Etopophos® Preservative Free
Chemotherapy
Fludarabine
Fludara
Chemotherapy
Gemcitabine hydrochloride
Gemzar®
Ibrutinib
Imbruvica
Ifosfamide
–
Lenalidomide
Revlimid
Leucovorin calcium
–
Mesna
Mesnex Injection
Methotrexate
–
Methylprednisolone;
Methylprednisolone sodium succinate
A-Methepred , Depo-Medrol ,
Medrol®, Solu-Medrol®
Steroid
Mitoxantrone hydrochloride
–
Chemotherapy
Oxaliplatin
Eloxatin®
Chemotherapy
Pentostatin
Nipent
Chemotherapy
Prednisone
–
Procarbazine hydrochloride
Matulane
Rituximab
Rituxan®
Targeted therapy
Vincristine sulfate
–
Chemotherapy
NCCN Guidelines for Patients®
Mantle Cell Lymphoma, Version 1.2016
®
Chemotherapy
Chemotherapy
®
Chemotherapy
Chemotherapy
®
®
Chemotherapy
Targeted therapy
®
Chemotherapy
Immunomodulator
®
Folic acid analog
Chemoprotectant
®
Chemotherapy
®
®
®
Steroid
®
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Chemotherapy
3
Overview of cancer treatments
Chemotherapy | Steroids
effects include another type of cancer, heart disease,
low levels of thyroid hormones (hypothyroidism), and
problems having babies (infertility).
Chemotherapy is given in cycles of treatment days
followed by days of rest. This allows your body to
recover before the next cycle. Cycles vary in length
depending on which drugs are used. Often, a cycle is
21 or 28 days long.
Not all side effects of chemotherapy are listed here.
Please ask your treatment team for a complete list of
common and rare side effects. If a side effect bothers
you, tell your treatment team. There may be ways to
help you feel better. There are also ways to prevent
some side effects.
A drug regimen may consist of one or more drugs.
When only one drug is used, it is called a single
agent. However, not all drugs work the same way,
so often more than one drug is used. A combination
regimen is the use of two or more drugs. Often, a
steroid, targeted therapy, or both are given along with
chemotherapy. Steroids and targeted therapy are
described later in this chapter.
Steroids
Part 4 is a guide that explains who should receive
which treatments. You will learn which regimens and
how many cycles may be part of your treatment.
Chemotherapy is sometimes given in high doses and
followed by stem cell transplant. Stem cell transplant
is described later in this chapter.
Steroids are a type of drug that is often used to
relieve inflammation. Steroids also are toxic to
lymphoma cells and therefore have strong anti-cancer
effects. Steroids used to treat mantle cell lymphoma
are listed in Chart 3.1, page 27. Steroid is the short
name for corticosteroids.
Side effects of chemotherapy
Steroids are a part of some chemotherapy
regimens. They are often given on the same days
as chemotherapy but only for a few days or a week.
Prednisone is made in pill form but dexamethasone
and methylprednisolone are made both as a liquid to
be injected or a pill to be swallowed.
The reactions to chemotherapy differ between people.
Some people have many side effects. Others have
few. Some side effects can be very serious while
others can be unpleasant but not serious. Most side
effects appear shortly after treatment starts and will
stop after treatment. However, other side effects are
long-term or may appear years later.
Most side effects of steroids fade away once the
drugs are stopped. Common side effects include
feeling hungry, trouble sleeping, mood changes, slow
wound healing, upset stomach, and swelling in the
ankles, feet, and hands.
Side effects of chemotherapy depend on many
factors. These factors include the drug type, amount
taken, length of treatment, and the person. In general,
most side effects are caused by the death of fastgrowing normal cells. These cells are found in the
blood, gut, hair follicles, and mouth. Thus, common
side effects of chemotherapy include low blood
cell counts, not feeling hungry, nausea, vomiting,
diarrhea, hair loss, and mouth sores. Lung damage
may also occur at the time of treatment. Late side
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Overview of cancer treatments
Targeted therapy
Targeted therapy
Targeted therapy describes a class of drugs that
stops the action of molecules that help cancer cells
grow. These drugs are less likely to harm normal cells
than chemotherapy. There are 2 targeted therapies
that are used to treat mantle cell lymphoma. These
treatments are briefly described next. Some side
effects are listed. Ask your treatment team for a full list
of common and rare side effects. In Part 4, information
on who should receive these drugs is provided.
Ibrutinib
Kinases are molecules that move chemicals, called
phosphates, from one molecule to another. Kinase
inhibitors stop the phosphate from being moved.
Kinase inhibitors often block growth signals within
cancer cells. This reduces the number of new cancer
cells being made.
to antigens. When antibodies are attached to antigens
on a cell, the cell is marked to be destroyed by your
immune system.
Rituximab is a monoclonal antibody that attaches to
CD20. See Figure 3.1. It works by marking cells for
destruction but it may directly kill the cells, too. It is a
very common treatment of mantle cell lymphoma.
Rituximab is a liquid that will be slowly injected into
your vein. It often takes a few hours to receive the full
dose. How often rituximab is received differs among
people. Ask your doctor for more information.
You may have an allergic reaction while receiving
rituximab. Other common side effects are chills,
infections, body aches, tiredness, and low blood cell
counts.
Figure 3.1 Anti-CD20 monoclonal antibody
Ibrutinib is a kinase inhibitor. It stops a kinase called
BTK (Bruton’s tyrosine kinase). This kinase is found
inside of normal B-cells and mantle cell lymphomas.
Anti-CD20 monoclonal antibodies attach to mantle
cell lymphoma cells to mark them for destruction by
your immune system.
Ibrutinib is a treatment option for mantle cell
lymphoma that wasn’t treated as planned or returned
after first-time treatment. It is made in pill form and
taken once a day around the same time. Your doctor
will tell you how many pills you need for your dose.
Common side effects of ibrutinib include diarrhea,
tiredness, muscle and bone pain, bruising, nausea,
upper respiratory tract infection, and rash. There may
be a short-lived increase in lymphocytes when first
taking ibrutinib. Serious but uncommon side effects
include bleeding, severe infections, heart and kidney
problems, and other cancers.
Rituximab
Derivative work of Rituximab Binding to CD20 on a B Cell Surface
by NIAID available at commons.wikimedia.org/wiki/File:Rituxima_
Binding_to_CD20_on_a_B_Cell_Surface_(6830897205).jpg under
a Creative Commons Attribution 2.0 Generic license
Monoclonal antibodies are man-made antibodies that
attach to proteins on cancer cells. The monoclonal
antibody used to treat mantle cell lymphoma attaches
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Overview of cancer treatmentsImmunomodulators | Stem cell transplant
Immunomodulators
Stem cell transplant
The immune system is your body’s natural defense
against infection and disease. Immunomodulators
are drugs that modify different parts of the immune
system. Lenalidomide is an immunomodulator that
may be received if first-time treatment does not work.
Hematopoietic stem cells are cells that develop
into mature blood cells. Hematopoietic stem cells
and mature blood cells are made in bone marrow.
Cancer or its treatment can damage or destroy the
cells in bone marrow. A stem cell transplant replaces
damaged or destroyed stem cells with healthy stem
cells, which form new marrow and blood cells. There
are two types of stem cell transplant.
Lenalidomide is made in pill form. It is given in cycles
of treatment days followed by days of rest. A cycle
may consist of 3 weeks of treatment and 1 week of
rest. It may also be given for 4 straight weeks. Cycles
may repeat until the cancer grows or side effects
become severe.
Autologous stem cell transplant uses your healthy
stem cells to repair bone marrow. This treatment
is also called HDT/ASCR (high-dose therapy with
autologous stem cell rescue). Healthy stem cells will
be collected from you when imaging tests show that
cancer treatment is working. You will then receive
intense chemotherapy and maybe radiation to destroy
any remaining cancer cells. This intense treatment
will also destroy bone marrow, so your healthy stem
cells will be put back into your body to “rescue” your
marrow.
Lenalidomide treats cancer in more than one way. As
an immunomodulator, it boosts the immune system.
It also helps stop cancer cells from increasing in
number. Third, it also works like a type of targeted
therapy called angiogenesis inhibitors. These drugs
stop the growth of new blood vessels that would
provide food (nutrients) to the cancer.
Allogeneic stem cell transplant uses healthy stem
cells that come from a donor. HLA (human leukocyte
antigen) typing is the test used to check if the donor
and your tissue type are a good fit. Chemotherapy will
be given to destroy cancer cells and suppress your
immune system from attacking the donor cells. The
transplanted stem cells will form new marrow and
attack remaining cancer cells. This attack is known
as the GVT (graft-versus-tumor) effect. On the other
hand, there is a serious risk of GVHD (graft-versushost disease). GVHD is when the donated cells see
the cells in your body as foreign and attack them.
Common side effects include low blood counts,
diarrhea, itching, rash, and extreme tiredness despite
sleep (fatigue). Serious but less common side effects
include blood clots, bleeding disorders, loss of vision,
and skin cancer. Ask your treatment team for a full list
of side effects.
A stem cell transplant is not an option for every
person with mantle cell lymphoma. A stem cell
transplant can have severe side effects so it is
not given to people who are frail or quite sick. An
autologous transplant is given only when prior drug
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Overview of cancer treatmentsStem cell transplant
treatment appears to have worked. An allogeneic
transplant may be an option after a second round of
drug treatment. Autologous stem cell transplant is
more commonly used for mantle cell lymphoma. More
details on this transplant are given next.
After apheresis or aspiration, the harvested cells
will be combined with a preservative. Then, they will
be frozen and stored to keep them alive until the
transplant. This process is called cryopreservation.
Collecting your stem cells
Before the autologous transplant, you will receive
high doses of chemotherapy. High doses are given
to kill any cancer cells that may remain after prior
treatment. Chemotherapy is often received for several
days. The transplant will occur 1 or 2 days later to
allow the chemotherapy to clear from your body.
Otherwise, the chemotherapy could damage the
healthy stem cells.
The first step of a autologous stem cell transplant
is to collect, or harvest, the blood stem cells. Blood
stem cells are found in the bone marrow and in the
bloodstream. If stem cells are collected from blood, a
process called apheresis will be done. First, medicine
is sometimes given to increase the number of stem
cells in blood. Then, some blood will be removed from
a large vein most likely in your arm. The blood will
flow through a tube and into a machine that removes
stem cells. The rest of the blood will be returned
through the other arm.
Apheresis typically takes 4 to 6 hours and does not
require anesthesia. It may take two or more sessions
to obtain enough stem cells. During the procedure,
you may have lightheadedness, chills, numbness
around the lips, and cramping in the hands.
Bone marrow aspiration is used to remove bone
marrow. For this procedure, either regional anesthesia
or general anesthesia will be given. Next, a needle
will be inserted through the skin into the hip bone
to draw out the bone marrow. The needle must be
inserted many times into one or more spots to collect
enough marrow. The marrow will then be processed
to collect the stem cells.
Collection of the bone marrow takes about 1 hour.
The entire hospital stay will likely be 6 to 8 hours,
which includes recovery time. The aspiration will
likely cause some pain and soreness for a few days.
Anesthesia may cause nausea, headache, and
tiredness.
NCCN Guidelines for Patients®
Mantle Cell Lymphoma, Version 1.2016
High-dose chemotherapy
Transplanting your stem cells
After chemotherapy, you will receive your healthy
stem cells through a transfusion. A transfusion is a
slow injection of blood products through a central
line into a large vein. A central line (or central venous
catheter) is a thin tube. The tube will be inserted into
your skin through one cut and into your vein through
a second cut. Local anesthesia is used. This process
can take several hours to complete.
The transplanted stem cells will travel to your bone
marrow and grow. New, healthy blood cells will form.
This is called engraftment. It usually takes about 2 to
4 weeks.
Until then, you will have little or no immune defense.
You will need to stay in a very clean room at the
hospital. You may be given an antibiotic to prevent or
treat infection. You may also be given a blood platelet
transfusion to prevent bleeding and blood transfusion
to treat low red blood counts (anemia). While waiting
for the cells to engraft, you will likely feel tired and
weak.
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Overview of cancer treatmentsRadiation therapy
Radiation therapy
used to keep you from moving. These may include a
mesh mask and body mold. You will be alone while
the therapists operate the machine from the nearby
control room.
Radiation therapy is sometimes used to treat stage I
and limited stage II cancers. Radiation therapy uses
high-energy rays to treat cancer. The rays damage
DNA. This either kills the cancer cells or stops new
cancer cells from being made. Radiation can also
harm normal cells. As a result, new methods keep
being made that target the tumor more precisely.
The therapists will be able to see, hear, and speak
with you. As treatment is given, you may hear noises.
One session takes less than 10 minutes. The types of
EBRT include:
External beam radiation therapy
• 3D-CRT (three-dimensional conformal
radiation therapy) – Treatment is completed
in about 6 weeks and uses photon beams that
match the shape of the tumor,
For mantle cell lymphoma, radiation is given using
a machine outside the body. This method is called
EBRT (external beam radiation therapy). ISRT
(involved-site radiation therapy) treats the lymph
nodes in which the cancer first started and cancer
near to these nodes. Radiation delivered to a larger
area is called IFRT (involved-field radiation therapy).
• IMRT (intensity-modulated radiation therapy)
– Treatment is completed in about 6 weeks
and uses photon beams of different strengths
based on the thickness of the tumor, and
Treatment planning with a simulation session is
needed. During simulation, pictures of the tumor will
be taken after your body is moved into the position
needed for treatment. CT with contrast is used. PET/
CT and MRI (magnetic resonance imaging) often
enhance treatment planning for ISRT. For tumors near
the breastbone, 4D-CT (four-dimensional computed
tomography) can account for tumor movement from
breathing. If your breathing causes large movements,
motion control methods during the scans may be
used.
• Proton therapy – Treatment is completed in
about 6 weeks and uses proton beams that
deliver radiation mostly within the tumor.
IGRT (image-guided radiation therapy) can improve
how well the radiation beam targets some tumors.
IGRT uses the machine that delivers the radiation
to also take images of the tumor and normal body
structures. This can be done right before or during
treatment. These images are compared to the ones
taken during simulation. If needed, changes will be
made to your body position or the radiation beams.
Using the scans, your treatment team will plan the
best radiation dose, number and shape of radiation
beams, and number of treatment sessions. Beams
are shaped with computer software and hardware
added to the radiation machine. Radiation beams are
aimed at the tumor with help from ink marks on the
skin or marker seeds in the tumor.
Side effects of radiation
Most side effects of radiation depend on where the
treatment was given. However, many people feel
fatigue. Changes in skin are also common right after
treatment. Your treated skin may look and feel as if
it has a mild sunburn. It may also become dry, sore,
and feel painful when touched. You may also have
short-term hair loss, but only where treated.
During treatment, you will lie on a table in the same
position as done for simulation. Devices may be
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Overview of cancer treatmentsRadiation therapy
Supportive care
Supportive care doesn’t aim to treat cancer
but aims to improve quality of life. It is
also called palliative care. It can address
many needs. One example is treatment
for physical and emotional symptoms.
Supportive care can also help with treatment
decisions as you may have more than one
option. It can also help with coordination
of care between health providers. Talk
with your treatment team to plan the best
supportive care for you.
Treatment to the head and neck can cause mouth
sores, dry mouth, changes in taste, and a sore throat.
Chest radiation can cause a dry cough or a sensation
of a lump when you swallow. Radiation near your
belly can cause nausea and maybe vomiting, and
when given between your hip bones, diarrhea and
cramps.
Not all side effects of radiation are listed here.
Please ask your treatment team for a complete list of
common and rare side effects. If a side effect bothers
you, tell your treatment team. There may be ways to
help you feel better. There are also ways to prevent
some side effects.
Late side effects of radiation may also occur. Again,
the effects depend on the treatment site. Examples
include dry mouth, dental cavities, hypothyroidism,
lung scarring, heart disease, infertility, and second
cancers.
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Overview of cancer treatments
Clinical trials
Clinical trials
Joining a clinical trial has benefits. First, you’ll have
access to the most current cancer care. Second, you
will receive the best management of care. Third, the
results of your treatment—both good and bad—will be
carefully tracked. Fourth, you may help other people
who will have cancer in the future.
New tests and treatments aren’t offered to the
public as soon as they’re made. They first need to
be studied. A clinical trial is a type of research that
studies a test or treatment.
Clinical trials study how safe and helpful tests and
treatments are. When found to be safe and helpful,
they may become tomorrow’s standard of care.
Because of clinical trials, the tests and treatments in
this book are now widely used to help people with
mantle cell lymphoma. Future tests and treatments
that may have better results than today’s treatments
will depend on clinical trials.
Clinical trials have risks, too. Like any test or
treatment, there may be side effects. Also, new tests
or treatments may not help. Another downside may
be that paperwork or more trips to the hospital are
needed.
To join a clinical trial, you must meet the conditions
of the study. Patients in a clinical trial are often alike
in terms of their cancer and general health. This is to
know that any progress is because of the treatment
and not because of differences between patients.
New tests and treatments go through a series of
clinical trials to make sure they’re safe and work.
Without clinical trials, there is no way to know if a
test or treatment is safe or helpful. Clinical trials have
four phases. Some examples of the four phases of
treatment are:
To join, you’ll need to review and sign a paper called
an informed consent form. This form describes the
study in detail. The study’s risks and benefits should
be described and may include others than those
described above.
• Phase I trials – aim to find the best dose of a
new drug with the fewest side effects.
Ask your treatment team if there is an open clinical
trial that you can join. There may be clinical trials
where you’re getting treatment or at other treatment
centers nearby. You can also find clinical trials
through the websites listed in Part 5.
• Phase II trials – assess if a drug works to
treat a specific type of cancer.
• Phase III trials – compare a new drug to the
standard treatment.
• Phase IV trials – test new drugs approved by
the U.S. FDA (Food and Drug Administration)
in many patients with different types of cancer.
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3
Overview of cancer treatments
Clinical trials
Complementary and alternative medicine
CAM (complementary and alternative medicine)
is a group of treatments that aren’t often given
by doctors. There is much interest today in
CAM for cancer. Many CAMs are being studied
to see if they are truly helpful.
Complementary medicines are treatments
given along with usual medical treatments.
While CAMs aren’t known to kill cancer cells,
they may improve your comfort and wellbeing. Two examples are acupuncture for pain
management and yoga for relaxation.
Alternative medicine is used in place of usual
medicine. Some alternative medicines are
sold as cures even though they haven’t been
proven to work in clinical trials. If there was
good proof that CAMs or other treatments
cured cancer, they would be included in this
book.
It is important to tell your treatment team if you
are using any CAMs. They can tell you which
CAMs may be helpful and which CAMs may
limit how well medical treatments work.
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3
Overview of cancer treatmentsReview
Review
• Chemotherapy stops the life cycle of cancer
cells so they can’t increase in number.
• A stem cell transplant rebuilds bone marrow
by giving a person healthy blood stem cells.
An autologous transplant uses your healthy
stem cells to rebuild marrow damaged by
high doses of chemotherapy that were given
to kill cancer cells. An allogeneic transplant
suppresses your immune system with
chemotherapy then transfuses donor stem
cells that will attack cancer cells.
• Some steroids have anti-cancer effects and
may be used with chemotherapy.
• Two targeted therapies are used to treat
mantle cell lymphoma. Ibrutinib stops some
growth signals within cancer cells. Rituximab
prompts your immune system to attack and
destroy cancer cells.
• Radiation kills cancer cells or stops new
cancer cells from being made.
• Lenalidomide is an immunomodulator. It treats
mantle cell lymphoma by modifying your
immune system and by other means.
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• Clinical trials give people access to new tests
and treatments that otherwise can’t usually
be received. These new tests and treatments
may in time be approved by the FDA.
36
4
Treatment guide
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4 Treatment guide
42
4.1 Watch and wait
Describes the few people who may
wait to have treatment.
44
4.2 Stage I and limited stage II
treatment
Lists treatment options for cancers
that are rarely found above or below
the diaphragm. If the cancer is in
more than one cluster of lymph
nodes, it is confined to a small area.
46
4.3 Extensive stage II and stage
III‒IV treatment
Lists treatment options for cancers
that involve a large area either above
or below the diaphragm, cancers that
are on both sides of the diaphragm,
or cancers that have widely spread
outside the lymphatic system.
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Review
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3
4
DCIS
Treatment
guideMantle
Genetic counseling
cell lymphoma
| Treatment
Part 4 is a guide to the treatment
options for people with mantle
cell lymphoma. This information is
taken from the treatment guidelines
written by NCCN experts of mantle
cell lymphoma. These treatment
guidelines list options for people with
mantle cell lymphoma in general.
Thus, your doctors may suggest
other treatment for you based on your
health and personal wishes. Fully
discuss your treatment options with
your doctor.
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guideMantle
Genetic counseling
cell lymphoma
| Treatment
Drug regimens for first-time treatment
Cycles 2, 4, and 6 consist of rituximab and highdose cytarabine.
Combinations of drugs are almost always used to
treat mantle cell lymphomas. These combination
regimens can be complex. Below, the first-time
regimens that are listed in Part 4 are briefly
described. Ask your treatment team for more
information.
RCHOP is given for up to 6 cycles. Each cycle
is 28 days long. RCHOP consists of rituximab,
cyclophosphamide, doxorubicin, vincristine, and
prednisone.
Bendamustine + rituximab, or BR, are given for
up to 6 cycles. Each cycle is 28 days long.
RCHOP/RDHAP is a 6-cycle regimen. Each
cycle is 21 days long. Cycles 1 and 2 consist of
CHOP (cyclophosphamide, doxorubicin, vincristine,
prednisone). Cycle 3 consists of RCHOP
(rituximab, cyclophosphamide, doxorubicin,
vincristine, prednisone). Cycles 4–6 consist of
RDHAP (rituximab, dexamethasone, cisplatin,
cytarabine).
CALGB 59909 is a series of treatments, which
includes:
• Two or three cycles of R-M-CHOP
(rituximab, methotrexate,
cyclophosphamide, doxorubicin, vincristine,
prednisone);
• One cycle of etoposide, cytarabine, and
rituximab;
• One cycle of high-dose carmustine,
etoposide, and cyclophosphamide followed
by autologous stem cell transplant;
• Rituximab maintenance weekly for two
weeks.
RCHOP/RICE is a 6- or 7-cycle regimen. Each
cycle is 21 days long. The first 4 cycles consist
of RCHOP (rituximab, cyclophosphamide,
doxorubicin, vincristine, prednisone). You may then
receive two or three cycles of RICE (rituximab,
ifosfamide, carboplatin, etoposide).
R-Hyper-CVAD is an 8-cycle regimen. Each
cycle is 21 days long. Cycles 1, 3, 5, and 7 consist
of rituximab, cyclophosphamide, vincristine,
doxorubicin, and dexamethasone. Cycles 2, 4, 6,
and 8 consist of rituximab, high-dose methotrexate,
and cytarabine.
Cladribine + rituximab are given for up to 6
cycles. Each cycle is 28 days long.
Modified R-Hyper-CVAD is given for up to 6
cycles. Each cycle is 28 days long. This regimen
is given only to people older than 65 years. No
methotrexate or cytarabine is received. Rituximab
maintenance is received weekly for 4 weeks then
repeated every 6 months for two years.
VR-CAP is an 8-cycle regimen. Each cycle is
21 days long. VR-CAP consists of bortezomib,
rituximab, cyclophosphamide, doxorubicin, and
prednisone.
NORDIC is a 6-cycle regimen. Each cycle
is 21 days long. Cycle 1 consists of maxiCHOP (dose-intensified cyclophosphamide,
vincristine, doxorubicin, prednisone) and cycles
3 and 5 consist of maxi-CHOP with rituximab.
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guideMantle
Genetic counseling
cell lymphoma
| Treatment
My notes
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Treatment
guide
Genetic
Watch
counseling
and wait| Treatment
4.1 Watch and wait
There are rare times when mantle cell lymphoma
does not need to be treated right away. Instead, a
“watch and wait” approach can be started. Watch
and wait is a period of close observation to see if the
cancer grows and treatment is needed.
Watch and wait may also be an option for slowgrowing mantle cell lymphoma. As described in Part
1, these cancers have mutations in the IGHV region
genes and often have low or absent SOX11 levels.
Watch and wait may be an option for mantle cell
lymphoma that isn’t causing symptoms. It is more
likely an option if you have a good performance score
and the cancer prognosis is fairly good.
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guide
Genetic
Watch
counseling
and wait| Treatment
My notes
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guideStage
Genetic counseling
I and limited
| Treatment
stage II treatment
4.2 Stage I and limited stage II treatment
Chart 4.2.1 First-time treatment
Treatment options
• Immunochemotherapy ± radiation therapy
•
• Less toxic treatments
◦◦ Bendamustine + rituximab
◦◦ VR-CAP
◦◦ Cladribine + rituximab
◦◦ RCHOP
◦◦ Modified R-Hyper-CVAD
More toxic treatments
◦◦ CALGB 59909
◦◦ R-Hyper-CVAD
◦◦ NORDIC
◦◦ RCHOP/RDHAP
◦◦ RCHOP/RICE
• Radiation therapy alone
Chart 4.2.2 Follow-up care
Type of care
Schedule of care
Medical history, physical exam, blood counts,
lactate dehydrogenase, metabolic panel
• Every 3–6 months for 5 years
◦◦ If normal results, then repeat every year or as needed
Chart 4.2.3 Refractory or relapse treatment
If first-time treatment was:
Your options are:
• Immunochemotherapy ±
radiation therapy
• Clinical trial
• Radiation therapy
• Drug treatment
◦◦ Bendamustine ± rituximab
◦◦ Bortezomib ± rituximab
◦◦ Cladribine + rituximab
◦◦ FC (fludarabine, cyclophosphamide) ± rituximab
◦◦ FCMR (fludarabine, cyclophosphamide, mitoxantrone, rituximab)
◦◦ FMR (fludarabine, mitoxantrone, rituximab)
◦◦ Ibrutinib
◦◦ Lenalidomide ± rituximab
◦◦ PCR (pentostatin, cyclophosphamide, rituximab)
◦◦ PEPC (prednisone, etoposide, procarbazine, cyclophosphamide)
± rituximab
• Radiation therapy alone
• Immunochemotherapy
◦◦ Without stem cell transplant, see Chart 4.3.1
◦◦ With stem cell transplant, see Chart 4.3.2
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guideStage
Genetic counseling
I and limited
| Treatment
stage II treatment
Chart 4.2.1 lists treatment options for stage I and
limited stage II cancers. These localized cancers
are rare. More research is needed to learn what
treatments are best.
For first-time treatment, two options are
advised by NCCN experts. The first option is
immunochemotherapy, which may be followed by
radiation therapy. The second option is radiation
therapy alone. Radiation therapy appears to be a
good treatment for limited mantle cell lymphomas.
For first-time treatment, immunochemotherapy
options are divided into two groups. Less toxic
treatments include drugs that are less likely to harm
your body than other drugs. Less toxic treatments
are for people who are: 1) 70 years of age and older;
and 2) younger than 70 years but have serious health
problems in addition to cancer. Some doctors use a
younger age to decide who can have more or fewer
toxic drugs. More toxic treatments include drugs that
can cause severe side effects but the cancer doesn’t
come back for some time. These drugs are for people
younger than age 70, who have good health other
than cancer.
Chart 4.2.2 lists follow-up care for when there are
no signs of cancer after treatment. This care may
include a medical history, physical exam, and blood
tests. A test schedule is listed in the chart but these
tests may be done whenever there are signs of
cancer. A biopsy is often needed to confirm there’s
cancer. If the cancer returns (relapse), options are
listed in Chart 4.2.3.
Chart 4.2.3 lists options for if first-time treatment
didn’t fully work or the cancer returned. Options
are based on your prior treatment. If you received
immunochemotherapy, you may have three options.
One option is a clinical trial of new drugs or of a stem
cell transplant. The second option is radiation therapy
if you didn’t receive it with immunochemotherapy.
The third option is a drug regimen listed in the chart,
and sometimes a regimen for diffuse large B-cell
lymphoma is tried.
Your first-time treatment may have consisted only
of radiation therapy. In this case, you can receive
immunochemotherapy. Treatment in Chart 4.3.1 does
not plan for a stem cell transplant, and in Chart 4.3.2,
a stem cell transplant is planned.
During and after cancer treatment, you may be
treated to prevent or control other health conditions.
Such actions are a part of supportive care. Health
conditions that are a concern for some people include
tumor lysis syndrome, reactivated viruses, and other
infections. Talk to your doctor about which health
conditions you may develop as a result of cancer
treatment.
At the end of treatment, you will receive an imaging
test to check the results. Imaging may be done with
CT or PET/CT. If there are no signs of cancer, you
may start follow-up care that is listed in Chart 4.2.2.
If there are signs of cancer, your options are listed in
Chart 4.2.3
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Genetic
Extensive
counseling
stage| IITreatment
and stage III‒IV treatment
4.3 Extensive stage II and stage III‒IV treatment
Chart 4.3.1 First-time treatment without stem cell transplant
Treatment
Test results
Treatment
• Clinical trial
• Bendamustine + rituximab
• VR-CAP
• Cladribine + rituximab
• RCHOP
• RCHOP/RICE
• Modified R-Hyper-CVAD
No signs of
cancer or cancer
looks smaller
• Clinical trial
• Consider rituximab maintenance
If the cancer looks the
same or larger, read
Part 4.3.4
Chart 4.3.2 First-time treatment with stem cell transplant
Treatment
Test results
Treatment
No signs of
cancer or cancer
looks smaller
• Clinical trial
• Clinical trial
• CALGB 59909
• R-Hyper-CVAD
• NORDIC
• RCHOP/RDHAP
• RCHOP/RICE
• Autologous stem
cell transplant
If the cancer looks the
same or larger, read
Part 4.3.4
Consider
rituximab
maintenance
Chart 4.3.3 Follow-up care
Type of care
Schedule of care
Medical history, physical exam, blood counts,
lactate dehydrogenase, metabolic panel
• Every 3–6 months for 5 years
◦◦ If normal results, then repeat every year or as needed
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guide
Genetic
Extensive
counseling
stage| IITreatment
and stage III‒IV treatment
Chart 4.3.1 lists options for immunochemotherapy
that isn’t followed by a stem cell transplant. These
options are for people who are 1) 70 years of age and
older; and 2) younger than 70 years but have serious
health problems in addition to cancer. Some doctors
use a younger age to decide who should receive
these drugs. A clinical trial is another option.
During and after cancer treatment, you may be
treated to prevent or control other health conditions.
Such actions are a part of supportive care. Health
conditions that are a concern for some people include
tumor lysis syndrome, reactivated viruses, and other
infections. Talk to your doctor about which health
conditions you may develop as a result of cancer
treatment.
CT or PET/CT will be used to assess the results of
immunochemotherapy. If the cancer is smaller or
can’t be seen, you may join a clinical trial or receive
rituximab. It is received every 8 to 12 weeks for 2
years or sometimes longer. If the cancer is the same
or worse after immunochemotherapy, options for
refractory treatment are listed in Chart 4.3.4.
CT or PET/CT will be used to assess the results of
immunochemotherapy. If the cancer is smaller or
can’t be seen, you may join a clinical trial or have an
autologous stem cell transplant. After the transplant,
you may re-start rituximab. It is received every 8 to 12
weeks for 2 years or sometimes longer. If the cancer
is the same or worse after immunochemotherapy,
options for refractory treatment are listed in Chart
4.3.4.
Chart 4.3.3 lists follow-up care for when there are
no signs of cancer after treatment. This care may
include a medical history, physical exam, and blood
tests. A test schedule is listed in the chart but these
tests may be done whenever there are signs of
cancer. A biopsy is often needed to confirm there’s
cancer. If the cancer returns (relapse), options are
listed in Chart 4.3.4.
Chart 4.3.2 lists options for immunochemotherapy
that is followed by a stem cell transplant. These
options are for people who are typically younger than
age 70 and who have good health other than cancer.
A clinical trial is another option.
During and after cancer treatment, you may be
treated to prevent or control other health conditions.
Such actions are a part of supportive care. Health
conditions that are a concern for some people include
tumor lysis syndrome, reactivated viruses, and other
infections. Talk to your doctor about which health
conditions you may develop as a result of cancer
treatment.
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Treatment
guide
Genetic
Extensive
counseling
stage| IITreatment
and stage III‒IV treatment
Chart 4.3.4 Refractory and relapse treatment
Treatment options
• Clinical trial
• Radiation therapy
• Drug treatment
◦◦ Bendamustine ± rituximab
◦◦ Bortezomib ± rituximab
◦◦ Cladribine + rituximab
◦◦ FC (fludarabine, cyclophosphamide) ± rituximab
◦◦ FCMR (fludarabine, cyclophosphamide, mitoxantrone, rituximab)
◦◦ FMR (fludarabine, mitoxantrone, rituximab)
◦◦ Ibrutinib
◦◦ Lenalidomide ± rituximab
◦◦ PCR (pentostatin, cyclophosphamide, rituximab)
◦◦ PEPC (prednisone, etoposide, procarbazine, cyclophosphamide) ± rituximab
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Genetic
Extensive
counseling
stage| IITreatment
and stage III‒IV treatment
Chart 4.3.4 lists options for if first-time treatment
didn’t work as planned or the cancer came back.
The best treatment approach is unknown, and more
research is needed. One option is to join a clinical
trial of new drugs or a stem cell transplant. A second
option is radiation therapy if you have not received
it before. The third option is a drug regimen listed in
the chart and sometimes a regimen for diffuse large
B-cell lymphoma is tried.
If the refractory or relapsed cancer is fully treated,
your doctor may suggest a stem cell transplant. An
allogeneic transplant is an option. You must be fairly
healthy to have this treatment.
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guideReview
Genetic counseling | Treatment
Review
• Treatment options for mantle cell lymphoma
are based on the cancer stage and your
health.
• During and after cancer treatment, you
may be treated to prevent or control other
health conditions. Such actions are a part of
supportive care.
• For stage I and limited stage II cancers,
options for first-time treatments are
immunochemotherapy with or without
radiation therapy and radiation therapy alone.
• Follow-up care is for cancers that were fully
treated. Your doctor will look for signs of
cancer growth. If tests suggest cancer growth,
more treatment will be needed.
• Joining a clinical trial is an option if you have
extensive stage II or stage III or IV cancer.
Besides a clinical trial, most of these cancers
are treated with immunochemotherapy alone.
If your body can endure severe side effects,
you may receive immunochemotherapy
followed by a stem cell transplant.
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• If immunochemotherapy doesn’t work as
planned or the cancer returns, there are
multiple treatment options. You may join a
clinical trial, receive radiation therapy if not
received before, or start a drug regimen that is
advised by NCCN experts.
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5
Making treatment decisions
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5 Making treatment
decisions
53It’s your choice
54
Questions to ask your doctors
58
Weighing your options
60Websites
60Review
Having cancer is very stressful.
While absorbing the fact that you
have cancer, you have to learn about
tests and treatments. In addition, the
time you have to accept a treatment
plan feels short. Parts 1 through 4
described the cancer and the test and
treatment options recommended by
NCCN experts. These options are
based on science and agreement
among NCCN experts. Part 5 aims
to help you make decisions that are
in line with your beliefs, wishes, and
values.
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5
Making treatment decisionsIt’s your choice
It’s your choice
The role patients want in choosing their treatment
differs. You may feel uneasy about making treatment
decisions. This may be due to a high level of stress.
It may be hard to hear or know what others are
saying. Stress, pain, and drugs can limit your ability to
make good decisions. You may feel uneasy because
you don’t know much about cancer. You’ve never
heard the words used to describe cancer, tests,
or treatments. Likewise, you may think that your
judgment isn’t any better than your doctors’.
Letting others decide which option is best may make
you feel more at ease. But, whom do you want to
make the decisions? You may rely on your doctors
alone to make the right decisions. However, your
doctors may not tell you which to choose if you have
multiple good options. You can also have loved
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ones help. They can gather information, speak on
your behalf, and share in decision-making with your
doctors. Even if others decide which treatment you
will receive, you still have to agree by signing a
consent form.
On the other hand, you may want to take the lead
or share in decision-making. Most patients do. In
shared decision-making, you and your doctors share
information, weigh the options, and agree on a
treatment plan. Your doctors know the science behind
your plan but you know your concerns and goals. By
working together, you are likely to get a higher quality
of care and be more satisfied. You’ll likely get the
treatment you want, at the place you want, and by the
doctors you want.
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5
Making treatment decisions
Questions to ask your doctors
Questions to ask your doctors
You will likely meet with experts from different fields of medicine. Strive to have helpful talks with
each person. Prepare questions before your visit and ask questions if the person isn’t clear. You can
also record your talks and get copies of your medical records. It may be helpful to have your spouse,
partner, or a friend with you at these visits. A patient advocate or navigator might also be able to come.
They can help to ask questions and remember what was said. Suggested questions to ask include:
What’s my diagnosis and prognosis?
It’s important to know that there are different types of cancer. Cancer can greatly differ even when
people have a tumor in the same organ. Based on your test results, your doctors can tell you which
type of cancer you have. He or she can also give a prognosis. A prognosis is a prediction of the pattern
and outcome of a disease. Knowing the prognosis may affect what you decide about treatment.
1. Where did the cancer start? In what type of cell?
2. Is this cancer common?
3. What is the cancer stage? Does this stage mean the cancer has spread far?
4. Is this a fast- or slow-growing lymphoma?
5. What other test results are important to know?
6. How often are these tests wrong?
7. Would you give me a copy of the pathology report and other test results?
8. Can the cancer be cured? If not, how well can treatment stop the cancer from growing?
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5
Making treatment decisions
Questions to ask your doctors
What are my options?
There is no single treatment practice that is best for all patients. There is often more than one
treatment option along with clinical trial options. Your doctor will review your test results and
recommend treatment options.
1. What will happen if I do nothing?
2. Can I just carefully monitor the cancer?
3. Do you consult NCCN recommendations when considering options?
4. Are you suggesting options other than what NCCN recommends? If yes, why?
5. Do your suggested options include clinical trials? Please explain why.
6. How do my age, health, and other factors affect my options?
7. Which option is proven to work best?
8. Which options lack scientific proof?
9. What are the benefits of each option? Does any option offer a cure? Are my chances any better
for one option than another? Less time-consuming? Less expensive?
10. What are the risks of each option? What are possible complications? What are the rare and
common side effects? Short-lived and long-lasting side effects? Serious or mild side effects?
Other risks?
11. What can be done to prevent or relieve the side effects of treatment?
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5
Making treatment decisions
Questions to ask your doctors
What does each option require of me?
Many patients consider how each option will practically affect their lives. This information may be
important because you have family, jobs, and other duties to take care of. You also may be concerned
about getting the help you need. If you have more than one option, choosing the option that is the
least taxing may be important to you:
1. Will I have to go to the hospital or elsewhere? How often? How long is each visit?
2. Do I have a choice of when to begin treatment? Can I choose the days and times of treatment?
3. How do I prepare for treatment? Do I have to stop taking any of my medicines? Are there foods I
will have to avoid?
4. Should I bring someone with me when I get treated?
5. Will the treatment hurt?
6. How much will the treatment cost me? What does my insurance cover? 7. Will I miss work or school? Will I be able to drive?
8. Is home care after treatment needed? If yes, what type?
9. How soon will I be able to manage my own health?
10. When will I be able to return to my normal activities?
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5
Making treatment decisions
Questions to ask your doctors
What is your experience?
More and more research is finding that patients treated by more experienced doctors have better
results. It is important to learn if a doctor is an expert in the cancer treatment he or she is offering.
1. Are you board certified? If yes, in what area?
2. How many patients like me have you treated?
3. How many procedures like the one you’re suggesting have you done?
4. Is this treatment a major part of your practice?
5. How many of your patients have had complications?
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5
Making treatment decisions
Weighing your options
Weighing your options
Deciding which option is best can be hard. Doctors
from different fields of medicine may have different
opinions on which option is best for you. This can be
very confusing. Your spouse or partner may disagree
with which option you want. This can be stressful. In
some cases, one option hasn’t been shown to work
better than another, so science isn’t helpful. Some
ways to decide on treatment are discussed next.
2nd opinion
very important decision. It can affect your length and
quality of life.
The time around a cancer diagnosis is very stressful.
People with cancer often want to get treated as soon
as possible. They want to make their cancer go
away before it spreads farther. While cancer can’t be
ignored, there is time to think about and choose which
option is best for you.
Support groups
Besides talking to health experts, it may help to talk
to patients who have walked in your shoes. Support
groups often consist of people at different stages of
treatment. Some may be in the process of deciding
while others may be finished with treatment. At
support groups, you can ask questions and hear
about the experiences of other people with mantle cell
lymphoma.
You may wish to have another doctor review your test
results and suggest a treatment plan. This is called
getting a 2nd opinion. You may completely trust your
doctor, but a 2nd opinion on which option is best can
help.
Compare benefits and downsides
Copies of the pathology report, a DVD of the imaging
tests, and other test results need to be sent to the
doctor giving the 2nd opinion. Some people feel
uneasy asking for copies from their doctors. However,
a 2nd opinion is a normal part of cancer care.
Every option has benefits and downsides. Consider
these when deciding which option is best for you.
Talking to others can help identify benefits and
downsides you haven’t thought of. Scoring each
factor from 0 to 10 can also help since some factors
may be more important to you than others.
When doctors have cancer, most will talk with more
than one doctor before choosing their treatment.
What’s more, some health plans require a 2nd opinion.
If your health plan doesn’t cover the cost of a 2nd
opinion, you have the choice of paying for it yourself.
If the two opinions are the same, you may feel more
at peace about the treatment you accept to have.
If the two opinions differ, think about getting a 3rd
opinion. A 3rd opinion may help you decide between
your options. Choosing your cancer treatment is a
NCCN Guidelines for Patients®
Mantle Cell Lymphoma, Version 1.2016
58
5
Making treatment decisions
Weighing your options
My notes
NCCN Guidelines for Patients®
Mantle Cell Lymphoma, Version 1.2016
59
3
4
DCIS
Treatment
guide
Genetic
Websites
counseling
| Review
| Treatment
1.1 H1 Here
Websites
Review
American Cancer Society
• Shared decision-making is a process in which
you and your doctors plan treatment together.
Body text here.
cancer.org/cancer/non-hodgkinlymphoma/
detailedguide/index
• Asking your doctors questions is vital to
getting the information you need to make
informed decisions.
National Coalition for Cancer Survivorship
www.canceradvocacy.org/toolbox
• Getting a 2nd opinion, attending support
groups, and comparing benefits and
downsides may help you decide which
treatment is best for you.
National Cancer Institute
cancer.gov/types/lymphoma
NCCN
nccn.org/patients
The Leukemia & Lymphoma Society (LLS)
LLS.org/informationspecialists
NCCN Guidelines for Patients®
Mantle Cell Lymphoma, Version 1.2016
60
Glossary
Dictionary
Acronyms
NCCN Guidelines for Patients®
Mantle Cell Lymphoma, Version 1.2016
61
GlossaryDictionary
Dictionary
allogeneic stem cell transplant
A cancer treatment that replaces blood stem cells with donor
stem cells which in turn make a new immune system and
attack the lymphoma.
cancer stage
Ratings of tumors that suggest the outlook of the disease.
chemotherapy
Drugs that stop the life cycle of cells so they don’t increase in
number.
anesthesia
Loss of feeling with or without loss of wakefulness that is
caused by drugs.
chromosome
Strands of genetic material inside of cells.
antibody
A protein made by white blood cells that helps fight off
infection. Also called an immunoglobulin.
chyle
A fatty liquid absorbed from the gut into the lymphatic
system.
antigen
Any substance that activates the immune system.
clinical trial
Research on a test or treatment to assess its safety or how
well it works.
autologous stem cell transplant
A cancer treatment that destroys cancer cells with high
doses of chemotherapy then rebuilds destroyed bone
marrow with your own healthy blood stem cells. Also called
an HDT/ASCR (high-dose therapy with autologous stem cell
rescue).
colonoscopy
Use of a thin, long tool that is guided into the colon to view or
remove tissue.
complete blood count (CBC)
A test of the number of blood cells in a sample.
B symptoms
Fevers, heavy night sweats, and weight loss without dieting
caused by B-cell cancers.
comprehensive metabolic panel
Tests of up to 14 chemicals in your blood.
B-cell
One of three types of a white blood cell called a lymphocyte.
computed tomography (CT)
A test that uses x-rays to view body parts.
beta-2 microglobulin
A small protein made by many types of cells.
contrast
A dye put into your body to make clearer pictures during
imaging tests.
biopsy
Removal of small amounts of tissue or fluid to be tested for
disease.
deoxyribonucleic acid (DNA)
A chain of chemicals inside cells that contains coded
instructions for making and controlling cells.
bone marrow
Soft, sponge-like tissue in the center of most bones where
blood cells are made.
diagnose
To identify a disease.
bone marrow aspiration
Removal of a small amount of bone marrow that is liquid to
test for disease.
differential
Measurement of the different types of white blood cells
present in a blood sample.
bone marrow biopsy
Removal of a small amount of solid bone and bone marrow
to test for disease.
echocardiogram
A test that uses sound waves to make pictures of the heart.
NCCN Guidelines for Patients®
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62
GlossaryDictionary
endoscopy
Use of a thin, long tool that is guided into the digestive track
to view or remove tissue.
immunochemotherapy
The use of both chemotherapy and other drugs that trigger a
response from the immune system.
external beam radiation therapy (EBRT)
Treatment with radiation that is delivered by a machine into
the body.
immunohistochemistry (IHC)
A test of cancer cells to find specific cell traits involved in
abnormal cell growth.
fatigue
Severe tiredness despite getting enough sleep that limits
one’s ability to function.
immunomodulator
A type of drug that modifies some parts of the body’s
disease-fighting system.
fertility specialist
An expert who helps men and women have babies.
intensity-modulated radiation therapy (IMRT)
Radiation therapy that uses small beams of different
strengths based on the thickness of the tissue.
flow cytometry
A test that looks at certain substances on the surface of cells
to identify the type of cells present.
fluorescence in situ hybridization (FISH)
A lab test that uses special dyes to look for abnormal
chromosomes.
four-dimensional computed tomography (4D-CT)
A CT scan that can show the movement of organs.
gastrointestinal (GI) tract
The group of organs through which food passes after being
eaten.
gene
Instructions in cells for making and controlling cells.
general anesthesia
A controlled loss of wakefulness from drugs.
germinal center
A short-lived structure that forms within a lymphatic organ in
response to germs.
involved-site radiation therapy (ISRT)
Treatment with high-energy rays (radiation) that is delivered
to lymph nodes and nearby sites with cancer.
karyotype
A test that uses a microscope to examine a cell’s
chromosomes.
kinase inhibitor
Cancer treatment that stops the transfer of phosphates,
which blocks growth signals to cancer cells.
lactate dehydrogenase
A protein that helps to make energy in cells.
liver
Organ that removes waste from the blood and helps to digest
food.
local anesthesia
A controlled loss of feeling in a small area of the body
caused by drugs.
human leukocyte antigen (HLA) typing
A blood test that finds a person’s unique set of proteins on
cells.
lumbar puncture
A procedure in which a thin needle is inserted between the
bones of the spine to remove a sample of spinal fluid or give
drugs into the spinal fluid.
image-guided radiation therapy (IGRT)
Radiation therapy that uses imaging tests during treatment to
better target the tumor.
lymph
A clear fluid containing white blood cells.
imaging test
A test that makes pictures (images) of the inside of the body.
lymph node
Small groups of special disease-fighting cells located
throughout the body.
immune system
The body’s natural defense against infection.
lymph vessel
Tube-shaped ducts that carry lymph throughout the body.
NCCN Guidelines for Patients®
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GlossaryDictionary
lymphatic system
Network in the body that collects and transports a fluid
(lymph) and fights germs.
radiation therapy
The use of radiation to treat cancer.
regional anesthesia
A type of drug used for short-term loss of feeling or
awareness in a part of the body without loss of wakefulness.
lymphocyte
A type of white blood cell that helps protect the body from
illness.
sedative
A drug that helps a person to relax or go to sleep.
lymphoma
Cancer that begins in white blood cells called lymphocytes
that are within the lymphatic system.
side effect
An unplanned physical or emotional response to treatment.
magnetic resonance imaging (MRI)
A test that uses a magnetic field and radio waves to make
pictures of the insides of the body.
spleen
An organ to the left of the stomach that helps protect the
body from disease.
mantle zone
An area within lymph nodes containing B-cells that can’t
make antibodies yet.
stem cell transplant
A cancer treatment that destroys bone marrow then replaces
it by adding healthy blood stem cells.
medical history
All health events and medications taken to date.
steroid
A drug used to reduce redness, swelling, and pain, but also
to kill cancer cells.
monoclonal antibody
Man-made antibodies that attach proteins on cancer cells.
supportive care
Treatment for the symptoms or health conditions caused by
cancer or cancer treatment.
multi-gated acquisition (MUGA) scan
A test of the heart that uses radiation to make pictures.
natural killer (NK) cell
One of three types of a white blood cell called a lymphocyte.
targeted therapy
Drugs that stop the growth process that is specific to cancer
cells.
pathologist
A doctor who’s an expert in testing cells to find disease.
T-cell
One of three types of a white blood cell called a lymphocyte.
performance status
A rating of one’s ability to do daily activities.
three-dimensional conformal radiation therapy
(3D-CRT)
Radiation therapy that uses beams that match the shape of
the tumor.
physical exam
A review of the body by a health expert for signs of disease.
positron emission tomography (PET)
A test that uses radioactive material to see the shape and
function of body parts.
thymus
A gland located behind the breastbone.
tonsil
A group of tissue within the throat that contains many white
blood cells called lymphocytes and fights germs that enter
the mouth and nose.
positron emission tomography/computed
tomography (PET/CT)
A test that uses radioactive material and x-rays to view the
shape and function of organs and tissues.
proton therapy
Radiation therapy that uses protons to treat a disease. Also
called hadron therapy.
NCCN Guidelines for Patients®
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GlossaryDictionary
translocation
The switching of parts between two chromosomes.
tumor lysis syndrome
A condition that occurs when many cancer cells die very
quickly and release their contents into the blood, which can
damage the kidneys and other organs.
ultrasound
A test that uses sound waves to take pictures of the inside of
the body.
uric acid
A chemical that is made and released into the blood when
cells and other substances in the body break down.
NCCN Guidelines for Patients®
Mantle Cell Lymphoma, Version 1.2016
65
GlossaryAcronyms
Acronyms
3D-CRT
three-dimensional conformal radiation therapy
HLA
human leukocyte antigen
BTK
Bruton’s tyrosine kinase
IGHV
immunoglobulin heavy-chain variable
CAM
complementary and alternative medicine
IGRT
image-guided radiation therapy
CBC
complete blood count
IHC
immunohistochemistry
CNS
central nervous system
IMRT
intensity-modulated radiation therapy
CT
computed tomography
IFRT
involved-field radiation therapy
DNA
deoxyribonucleic acid
ISRT
involved-site radiation therapy
EBRT
external beam radiation therapy
MRI
magnetic resonance imaging
ECOG
Eastern Cooperative Oncology Group
MUGA
multi-gated acquisition
FDA
Food and Drug Administration
NK cells
natural killer cells
FISH
fluorescence in situ hybridization
PET
positron emission tomography
FNA
fine-needle aspiration
TLS
tumor lysis syndrome
GI
gastrointestinal
NCCN Abbreviations and Acronyms
GVHD
graft versus host disease
NCCN®
National Comprehensive Cancer Network®
GVT
graft versus tumor
NCCN Patient Guidelines
NCCN Guidelines for Patients®
HDT/ASCR
high-dose therapy with autologous stem cell rescue
NCCN Guidelines for Patients®
Mantle Cell Lymphoma, Version 1.2016
NCCN Guidelines®
NCCN Clinical Practice Guidelines in Oncology®
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NCCN Guidelines for Patients®
Mantle Cell Lymphoma, Version 1.2016
68
NCCN Panel Members
NCCN Panel Members for
Non-Hodgkin’s Lymphomas
Andrew D. Zelenetz, MD, PhD/Chair
Memorial Sloan Kettering Cancer Center
Leo I. Gordon, MD/Co-Vice Chair
Nancy Lee Harris, MD
Massachusetts General Hospital
Cancer Center
Robert H. Lurie Comprehensive Cancer
Center of Northwestern University
Francisco Hernandez-Ilizaliturri, MD
William G. Wierda, MD, PhD/CoVice Chair
Richard T. Hoppe, MD
The University of Texas
MD Anderson Cancer Center
Jeremy S. Abramson, MD
Massachusetts General Hospital
Cancer Center
Ranjana H. Advani, MD
Stanford Cancer Institute
Roswell Park Cancer Institute
Stanford Cancer Institute
Steven M. Horwitz, MD
Nishitha Reddy, MD
Vanderbilt-Ingram Cancer Center
Erin Reid, MD
UC San Diego Moores Cancer Center
Kenneth Roberts, MD
Yale Cancer Center/Smilow Cancer Hospital
Ayman A. Saad, MD
Memorial Sloan Kettering Cancer Center
University of Alabama at Birmingham
Comprehensive Cancer Center
Mark S. Kaminski, MD
Lubomir Sokol, MD, PhD
University of Michigan
Comprehensive Cancer Center
Christopher R. Kelsey, MD
Moffitt Cancer Center
Lode J. Swinnen, MB, ChB
Duke Cancer Institute
The Sidney Kimmel Comprehensive
Cancer Center at Johns Hopkins
UCSF Helen Diller Family
Comprehensive Cancer Center
Youn H. Kim, MD
Julie M. Vose, MD, MBA
Nancy Bartlett, MD
Susan Krivacic, MPAff
Lynn Wilson, MD, MPH
Ann S. LaCasce, MD
Joachim Yahalom, MD
C. Babis Andreadis, MD, MSCE
Siteman Cancer Center at BarnesJewish Hospital and Washington
University School of Medicine
John C. Byrd, MD
The Ohio State University Comprehensive
Cancer Center - James Cancer Hospital
and Solove Research Institute
Luis E. Fayad, MD
The University of Texas
MD Anderson Cancer Center
Richard I. Fisher, MD
Fox Chase Cancer Center
Martha J. Glenn, MD
Huntsman Cancer Institute
at the University of Utah
Thomas M. Habermann, MD
Mayo Clinic Cancer Center
Stanford Cancer Institute
Patient Advocate
Dana-Farber/Brigham and
Women’s Cancer Center
Matthew Lunning, DO
Fred & Pamela Buffett Cancer Center
Auayporn Nademanee, MD
City of Hope Comprehensive Cancer Center
Pierluigi Porcu, MD
The Ohio State University Comprehensive
Cancer Center - James Cancer Hospital
and Solove Research Institute
Oliver Press, MD, PhD
Fred Hutchinson Cancer Research Center/
Seattle Cancer Care Alliance
Rachel Rabinovitch, MD
University of Colorado Cancer Center
For disclosures, visit www.nccn.org/about/disclosure.aspx.
NCCN Guidelines for Patients®
Mantle Cell Lymphoma, Version 1.2016
69
Fred & Pamela Buffett Cancer Center
Yale Cancer Center/Smilow Cancer Hospital
Memorial Sloan Kettering Cancer Center
Nadeem Zafar, MD
St. Jude Children’s Research Hospital/
University of Tennessee
Health Science Center
NCCN Member Institutions
NCCN Member Institutions
Fred & Pamela Buffett Cancer Center
Omaha, Nebraska
800.999.5465
nebraskamed.com/cancer
Case Comprehensive Cancer Center/
University Hospitals Seidman
Cancer Center and Cleveland Clinic
Taussig Cancer Institute
Cleveland, Ohio
800.641.2422 • UH Seidman Cancer Center
uhhospitals.org/seidman
866.223.8100 • CC Taussig Cancer Institute
my.clevelandclinic.org/services/cancer
216.844.8797 • Case CCC
case.edu/cancer
City of Hope Comprehensive
Cancer Center
Los Angeles, California
800.826.4673
cityofhope.org
Dana-Farber/Brigham and
Women’s Cancer Center
Massachusetts General Hospital
Cancer Center
Boston, Massachusetts
877.332.4294
dfbwcc.org
massgeneral.org/cancer
Duke Cancer Institute
Durham, North Carolina
888.275.3853
dukecancerinstitute.org
Fox Chase Cancer Center
Philadelphia, Pennsylvania
888.369.2427
foxchase.org
Huntsman Cancer Institute
at the University of Utah
Salt Lake City, Utah
877.585.0303
huntsmancancer.org
Fred Hutchinson Cancer
Research Center/
Seattle Cancer Care Alliance
Seattle, Washington
206.288.7222 • seattlecca.org
206.667.5000 • fredhutch.org
The Sidney Kimmel Comprehensive
Cancer Center at Johns Hopkins
Baltimore, Maryland
410.955.8964
hopkinskimmelcancercenter.org
Robert H. Lurie Comprehensive Cancer
Center of Northwestern University
Chicago, Illinois
866.587.4322
cancer.northwestern.edu
Mayo Clinic Cancer Center
Phoenix/Scottsdale, Arizona
Jacksonville, Florida
Rochester, Minnesota
800.446.2279 • Arizona
904.953.0853 • Florida
507.538.3270 • Minnesota
mayoclinic.org/departments-centers/mayoclinic-cancer-center
Stanford, California
877.668.7535
cancer.stanford.edu
University of Alabama at Birmingham
Comprehensive Cancer Center
Birmingham, Alabama
800.822.0933
www3.ccc.uab.edu
UC San Diego Moores Cancer Center
La Jolla, California
858.657.7000
cancer.ucsd.edu
UCSF Helen Diller Family
Comprehensive Cancer Center
San Francisco, California
800.689.8273
cancer.ucsf.edu
Memorial Sloan Kettering
Cancer Center
University of Colorado Cancer Center
Moffitt Cancer Center
University of Michigan
Comprehensive Cancer Center
New York, New York
800.525.2225
mskcc.org
Tampa, Florida
800.456.3434
moffitt.org
Aurora, Colorado
720.848.0300
coloradocancercenter.org
Ann Arbor, Michigan
800.865.1125
mcancer.org
The Ohio State University
Comprehensive Cancer Center James Cancer Hospital and
Solove Research Institute
Columbus, Ohio
800.293.5066
cancer.osu.edu
The University of Texas
MD Anderson Cancer Center
Houston, Texas
800.392.1611
mdanderson.org
Vanderbilt-Ingram Cancer Center
Roswell Park Cancer Institute
Buffalo, New York
877.275.7724
roswellpark.org
Siteman Cancer Center at BarnesJewish Hospital and Washington
University School of Medicine
St. Louis, Missouri
800.600.3606
siteman.wustl.edu
Nashville, Tennessee
800.811.8480
vicc.org
University of Wisconsin
Carbone Cancer Center
Madison, Wisconsin
608.265.1700
uwhealth.org/cancer
Yale Cancer Center/
Smilow Cancer Hospital
St. Jude Children’s Research Hospital/
The University of Tennessee
Health Science Center
Memphis, Tennessee
888.226.4343 • stjude.org
901.683.0055 • westclinic.com
NCCN Guidelines for Patients®
Mantle Cell Lymphoma, Version 1.2016
Stanford Cancer Institute
70
New Haven, Connecticut
855.4.SMILOW
yalecancercenter.org
Notes
My notes
NCCN Guidelines for Patients®
Mantle Cell Lymphoma, Version 1.2016
71
IndexMantle cell lymphoma
Index
biopsy 10, 14, 17, 21, 24, 45, 47
blood tests 18, 24, 45, 47
chemotherapy 17–18, 26–31, 36
clinical trial 34, 36, 44–50, 55
complementary and alternative medicine 35
complete blood count 17–18
comprehensive metabolic panel 17–18
computed tomography 19, 32
diagnosis 4, 10, 21, 54, 58
fertility 23, 24
immunohistochemistry 10
immunomodulators 30
involved-site radiation therapy 32
lymphatic system 6–8, 14, 38
lymphocyte 7–8, 14, 29
lymphoma 6, 8, 10–12, 14, 18, 28–29, 45, 49, 54, 58
medical history 16, 17, 44–47
NCCN Member Institutions 70
NCCN panel members 69
pathology report 10, 54, 58
physical exam 17, 45–47
positron emission tomography/computed
tomography 19
radiation therapy 32, 44, 45, 48–50
stem cell transplant 30–31, 36, 40, 44–47, 49–50
steroids 28, 36
supportive care 33, 45, 47, 50
targeted therapy 29–30
NCCN Guidelines for Patients®
Mantle Cell Lymphoma, Version 1.2016
72
Ü
NCCN Guidelines for Patients®
NCCN Non-HodGkin’s LymphomaS Series
Mantle Cell
Lymphoma
Version 1.2016
NCCN Foundation® gratefully acknowledges our industry supporters Gilead Sciences, Pharmacyclics, LLC an AbbVie Company,
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