Download Leukemia

Rita All
Carrie Warner
Leukemia is a group of malignant diseases that
result in changes to circulating lymphocytes
characterized by diffuse, abnormal growth of
leukocytic precursors in the marrow.
 The uncontrolled increase in immature white
blood cells suppresses normal hematopoietic stem
cells leading to anemia and thrombocytopenia.
 Causes life threatening infections due to ineffective
WBC function.
 Classification is made by the course of the illness
and types of cells and tissues involved.
 Staging is not possible as it is already circulating.
(Burns, 2013; Larson, 2014).
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(American Cancer Society, 2014)
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According to the American Joint Committee on Cancer
(AJCC)
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Circulating cells= leukemia
Tumor masses in lymph nodes and tissues= lymphoma
If both= lymphoma/leukemia
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Emerging research suggests these are the same diseases in
different stages
 Chronic lymphocytic leukemia= small lymphocytic lymphoma (a form
of non-Hodgkins lymphoma)
 If greater than 25% of marrow replaced by cancerous lymphocytes, it is
usually considered leukemia.
 Also if the lymph nodes are very large, diagnosis will generally be
lymphoma.
(American Cancer Society, 2014)
Normal bone marrow elements are replaced with blast cells
that are abnormal and poorly differentiated.
 The abnormal cells (Lymphoblasts or Myeloid cells) take
over and cause unregulated clonal proliferation of
malignant cells, crowding out other healthy cell groups.
 Chronic lymphocytic leukemia progresses slowly and can
last decades.
 People with Chronic Myeloid leukemia acquired a
chromosomal abnormality that causes part of one
chromosome to break off and join another, the short
chromosome called the Philadelphia chromosome, which
produces an enzyme that causes WBCs to grow out of
control, that can ultimately result in acute leukemia.
(Larson, 2014)

(National Cancer Institute,
2013)
Unknown etiology for all types, but higher
incidences with some that have:
 Infection
 Radiation
 Chemical and Drug exposure
 Epstein- Barr may play a role in in Burkitt
leukemia/ lymphoma.
 Genetic mutations may lead to disruption of
lymphocytes and prolonged survival.
(Domino, 2014; Burns, 2013; National Cancer
Institute, 2013).
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There were 48,610 new cases of leukemia in 2013, 2.9% of all cancer cases.
There were 23,720 deaths in 2013 from leukemia, 4.1% of all cancer deaths.
The five year survival rate for leukemia is 56%
Leukemia is the most common form of childhood cancer, 41% of pediatric
malignancies in children less than 15 (Burns, 2013).
Four main types of leukemias, with many rare varants and subtypes.
ALL- In the US- 1,000 adult cases per year, median age 35-40 years old,
incidence increases with age, males slightly more prevalent than females.
Accounts for 80% of all childhood leukemia cases, peak age 2-6 years old.
Occurs one out of every 29,000 children annually (Burns, 2013).
AML- Increases with age, median age 70 years. 13,500 cases in 2007, second
most common type of leukemia for adults and children. Affects males
slightly more than females.
CLL- 15,000 to 17,000 new cases reported annually. Most common form of
leukemia in adults. Median age of diagnosis is 70 years, with rising
incidence in those greater than 55 years old. Males have higher incidence
1.7:1, and higher with Caucasian than African Americans.
CML- 1.6 cases per 100,000 annually. Greatest in 50-60 year olds, with males
having higher incidence 1.3:1. Accounts for 15-20 % of adult leukemias
(Burns, 2013; Domino, 2014)
(Lee, 2009)
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There is no individual screening test for Leukemias.
Those with risk factors can be monitored more
diligently with routine exams and labs.
Routine or illness physical: Presence of organomegalyhepatosplenomegaly , enlarged lymph nodes.
CBC with Differential (Often found during routine
exam or illness)
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Low or high white blood cells, varying levels of neutropenia
Thrombocytopenia present in 85 % of cases
Peripheral smear- malignant cells
Low hemoglobin, less than 9.0
Liver Function Tests may show elevations
(Burns, 2013)
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ALL- Less than 15 years old, greater than 60 years old. Chemical
exposure to benzene or radiation. Can follow aplastic anemia.
AML- Genetic predisposition (Down’s syndrome, Bloom’s
syndrome), Fanconi anemia, neurofibromatosis, Li- Fraumeni
Syndrome, Wiskott- Aldrich syndrome, Kostmann syndrome, and
Diamond- blackfan anemia. Radiation exposure, Immunodeficiency
states, Chemical and drug exposure (nitrogen mustard, benzene),
Myelodysplastic syndromes, Cigarette smoking (20%).
CLL- Mostly uncertain risk factors, possible chronic immune
stimulation.
CML- Ionizing radiation exposure
All leukemias: possibly other causes such as workplace exposures
from organic solvents, pesticides, herbicides, hair dyes;
electromagnetic fields, birth weight over 7.7 lbs, mom over 35 yrs at
birth, and parent exposures.
(Domino, 2014).
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Children:
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Signs and symptoms related to
leukemic replacement of bone
marrow/ absence of blood cell
precursors
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Anemia, Pallor, listlessness
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Irritability, chronically tired
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History of repeated infections, fever,
weight loss
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Bleeding- epistaxis, petechiae,
hematomas
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Lymphadenopathy and
hepatosplenomegaly
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Bone and joint pain
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CNS symptoms if brain involvement
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Abnormal CBC, low or high white
cell count with out of proportion
differential
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Testicular pain
(Burns, 2013)
Adults:
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Acute forms have bone and joint
pain
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Gingival hyperplasia with bleeding
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S/S infection- Fevers, chills,
palpitations, shortness of breath
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Skin eruptions, easy bruising,
prolonged bleeding times

Chronic forms c/o fatigue, night
sweats, low grade fevers
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CML- leukostasis- blurred vision,
respiratory distress, priapism
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Organomegaly with chronic form,
hepatosplenomegaly with enlarged
spleen and lymph nodes, assoc with
N/V
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Pallor, Anemia
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Abnormal CBC, low or high white
cell count with out of proportion
differential
(Dunphy, 2011)
Acute Lymphocytic Leukemia

AML

CML in Lymphoid blast phase

Prolymphocytic leukemia

Malignant Lymphomas

Multiple Myeloma

Bone marrow metastases from solid
tumors (breast, prostate, lung, renal)

Myelodysplastic syndromes
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Aplastic anemia
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Myelofibrosis
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Autoimmune disease (lupus, felty
syndrome)
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Infectious mononucleosis
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Pertussis
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Autoimmune Thrombocytopenia
purpura
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Leukemoid reaction to infection
(Domino, 2014).
Acute Myeloid Leukemia

Virus Induced Cytopenia,
lymphadenopathy, and
organomegaly
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Immune Cytopenias
(including Systemic lupus
erythmatosis)

Drug induced cytopenias
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Other marrow failure and
infiltrative disease (aplastic
anemia, paroxysmal
nocturnal hemoglobinuria,
myelodysplastic syndromes,
Gaucher disease)
(Domino, 2014).
Chronic Myelogenous Leukemia

Chronic Myelomonocytic
leukemia
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Chronic neutrophilic leukemia
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Chronic eosinophilic leukemia
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Juvenile myelomonocytic
leukemia
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Infectious mononucleosis
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Leukemoid reaction
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Polycythemia vera
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Treatment with granulocyte
stimulating factors
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Acute myelogenous leukemia
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Acute lymphoblastic leukemia
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Atypical CML
(Domino, 2014).
Chronic Lymphocytic Leukemia

Bacterial Tuberculosis

Infectious mononucleosis

Non hodgkins Lymphoma

Hairy cell leukemia

Waldenstrom
macroglobulinemia

Large granular lymphocytic
leukemia
(Domino, 2014).
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For children:
 No physical activities
 Limited exposure to other
children and family members
due to risk of contracting
infection.
 Frequent hospitalizations for
treatments, and follow ups.
 Frequent illness due to disease
and treatment modalities.
 Changing of physical self, loss of
hair, swelling, pale skin and
dark circles under eyes, weight
loss, central lines or ports.
 Limited exposure to animals.
 Family pressures with medical
bills and transportation.
 Threat of mortality.
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For adults:
 Frequent monitoring,
hospitalizations for testing,
treatments, and follow up.
 Missed work, or need for leave
of absence.
 Loss of income due to missed
work time, leave of absence, and
elevated medical bills.
 Family dynamic changes, the
primary caregiver may become
the one being cared for.
 Changing of physical self, loss of
hair, swelling, pale skin and
dark circles under eyes, weight
loss, central lines or ports.
 Family pressures due to
decreased income, increased
bills, changes in family
dynamics, and feeling of lack of
self worth.
 Threat of mortality.
(Dunphy, 2011; Larson, 2014)
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CBC with Manual Differential
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Uric acid level
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Elevation
Bone marrow Bx/ aspiration
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Elevation
CRP
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Elevation due to inflammation of liver
Sed Rate
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Presence of biomarkers in blood/ bone marrow cells . DNA abnormality marker with AML or
CML “philadelphia chromosome”
Liver Function Tests
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Elevated
PCR (polymerase chain reaction)
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Can be elevated or high
LDH
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Low or high white blood cells, varying levels of neutropenia
Thrombocytopenia present in 85 % of cases
Peripheral smear- malignant cells
Low hemoglobin, less than 9.0
Replacement of normal marrow cells by blast cells
Should parallel peripheral smear
Lumbar Puncture, CXR, CT scans, MRIs- to rule out other pathology, CNS involvement,
hepatosplenomegaly
(Burns, 2013)
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Treatment varies on
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Type of leukemia
 If chronic, usually asymptomatic, immediate treatment may
not be required
 Goal is for regular checkups, control disease and symptoms,
and is seldom curable.
 If acute, treatment begins right away
 Goal is Remission, prevention of relapse, and it can be curable.
Extent of disease
 If has been treated for cancer before
 Age, symptoms, general health
Maintaining health physically and mentally
(National Cancer Institute, 2013).
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Bone marrow transplant
 Splenectomy
 Radiation
 Clinical trials
 Nutritional support (IV fluids/ hyperalimentation)
 Avoidance of antiplatelets (ASA)
 Neutropenic guidelines/ reverse isolation for
infection prevention
 Blood transfusions
(Domino, 2014)
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ALL- 4 phases of treatment
 Remission induction chemotherapy
One month
Kill as many cancer causing cells as
possible
Corticosteroids
Transfusions
goal- Induce remission
 Consolidation, or CNS prophylaxis
Preventative therapy
Stops spread to brain/ spinal cord
High Dose IV or Intrathecal chemo
Radiation to the brain PRN
Usually lasts 2-6 months
 Intensification therapy
Starts when in remission
high dose chemo to kill any lingering
cells1-2 times, 1-2 months each time
 Maintenance therapy
Chemo for several years to remain in
remission
Girls- 2 years, Boys- 3 years
AML- Intermediate and high risk patients
curative potential only from Bone marrow
transplant.
•Transretinoic acid and arsenic trioxide
promote maturation to granulocytes.
•Idarubicin is used for induction therapy in 3-4
cycles.
CLL- Most patients do not require active
treatment.
•Early treatment is not advised.
•High risk patients are only treated.
•Three main groups of drugs: (COP or CHOP
treatment- cyclophosphamide, vincristine,
prednisone, doxorubicin)
1. Alkylating agents- chlorambusil,
bendamustine, cyclophosphamide
2. Purine analogues- fludarabine,
pentostatin
3. Monoclonal antibodiesrituximab, alemtuzumab
CML- Bone marrow transplant is the only
known cure. TKI’s (imatinib) provide long
term control of disease, no immature blood
cells, and no Philadelpha positive metaphases.
(Katzung, Masters, & Trevor, 2012).
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Chemotherapeutics- common toxic side effects include nausea,
vomiting, immuno and myelosuppression. Can also cause hepato ,
nephro, and neurotoxicity. They are often used in multiples.
Alkylating agents- DNA synthesis and function inhibition, as well as
mitosis inhibition and catastrophe, leading to cell death. (bendamustine,
Cyclophosphamide, Busulfan)
 Antimetabolites- Inhibit DNA synthesis and repair (Cytarabine,
Fludarabine, Cladribine, 6-MP, 6- Thioguanine, methotrexate)
 Antitumor antibiotics- oxygen free radicals bind to DNA and RNA
breaking it and interfering with replication. (Daunorubicin, Idarubicin)
 Asparginase- enzyme isolated from E coli, hydrolyzes L asparagine,
causing rapid inhibition of protein synthesis.
 Imatinib- Inhibits tyrosine kinase in Philadephia chromosome related
CML.
Corticosteriods- used in conjunction with Chemotherapeutics to decrease
inflammation, immunosuppression, and further chances for remission.
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(Katzung, Masters, & Trevor, 2012).
Infection
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Anemia
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Excess bleeding/ bruising (low PLT)
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Clotting disorders ( elevated PLT), DVT, CVA
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Richter transformation- 3-5% of patients with CLL develop diffuse large B cell
lymphoma, and prognosis is generally poor.
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Other cancer developments due to cancer or treatments- Kaposi Sarcoma, Melanoma,
Bladder, Lung, stomach, throat cancers.
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Hairy cell leukemia- can develop hodgkins, non- hodgkins, thyroid ca.
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Kidney failure (Rare)
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Ruptured spleen (due to enlargement)
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CNS involvement- seizures, headache, vomiting, confusion, loss of coordination
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Depression
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Pain (increased WBC in marrow)
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Infertility
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Death
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Therapy related illness, including avascular necrosis, transfusion reaction, neurotoxicity,
tumor lysis syndrome,
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Late effects (66%)- learning disabilities, poor work performance, psyche distress, health
insurance discrimination, “chemo brain”- inability to concentrate.
(Dunphy, 2011; Burns, 2013)
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Chronic leukemias
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Recommended check ups every 6 months
Acute leukemias
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Every month after treatment for the first year
To monitor for health changes and treat as
necessary
 Monitor for return of cancer or the worsening of
chronic Leukemias
 Physical exam, blood tests, bone marrow tests,
other tests as indicated.
(National Cancer Institute, 2013).
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Support groups for patient and family can be helpful with
resources, education and support. Immunizations need to
be up to date ie: flu, pneumovac
 At time of diagnosis: How to care for yourself before
treatment, avoidance of infection, rest, planning.
 During therapy: Managing your symptoms, sexual changes
and prevention of pregnancy, preventing and treating
infections, preventing clotting, bleeding, staying active,
relaxation, limiting visitors without isolation, coping with
stressors.
 After treatment: Keeping follow up appointments,
normalizing life again, monitoring for infections, “late
effects”
 Stop smoking!
(National Cancer Institute, 2013).
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Hematologist
Medical Oncologist
Pediatric oncologist
Radiation oncologist
Oncology nurse
Social worker
Registered dietician
Gastroenterologist,
Nephrologist, Neurologist as
needed
Cancer Centers *
* Cancer centers are recommended
for Leukemias, especially acute
forms
Gynecologist
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Complementary therapists:
Acupuncture, Reiki, Meditation,
Hypnosis, Aromatherapist
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Hospice
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Pain management
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Cancer rehabilitation
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Home care
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Psychiatrist, counselors
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Physical therapy
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Spiritual/ Religious counselors
(National Cancer Institute, 2013).
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1. True or false? The goal of acute Leukemia is remission.
2. True or false? The etiology of most types of leukemia is
unknown.
3. What is the median age for AML and CLL?
a.) 30
b.) 50
c.) 10
d.) 70
4. What is the most common type of leukemia in children?
a.) ALL
b.) AML
c.) CML
d.) CLL
5. A patient presents to the office with newly diagnosed Chronic lymphocytic leukemia.
They want to know when their therapy will begin. You tell them:
a.) Treatment begins right away
b.) Early treatment is not recommended for those patients not at high risk
c.) Treatment begins when the patient is infection free
6. What types of leukemia would a Bone marrow transplant be the only curative measure?
a.)
b.)
c.)
d.)
ALL and CLL
AML and CML
ALL and AML
CLL and CML
7. A patient comes into the office and is concerned about the staging of their leukemia. You
tell them:
a.) Blood cancers are already widespread, so there is no staging like tumor related cancers.
b.) Their cancer is staged based on their age.
c.) Their cancer is based on the amount of symptoms they are currently experiencing.
8. The difference between lymphoma and leukemia is:
a.) Leukemia occurs in the white blood cells and lymphoma occurs in the
marrow
b.) Leukemia occurs in the white blood cells and lymphoma occurs in the
lymph tissue
c.) The two are exactly the same.
9. A 32 year old female who has been in remission for 5 years with ALL comes
to the office because she has not been able to get pregnant. You tell her:
a.) Keep trying, it will happen.
b.) She should not ever have children due to the risk of relapse.
c.) She may have infertility issues due to her treatments, and should see
an OB/GYN right away.
10. A 42 year old who was recently diagnosed with AML. Which of the
following would least likely be a risk factor for this type?
a.) gender
b.) smoking
c.) chemical exposure
d.) family history
American Cancer Society. (2014). Leukemia – Acute Lymphocytic (Adults).
Retrieved from
http://www.cancer.org/acs/groups/cid/documents/webcontent/003109pdf.pdf.
Burns, C. (2013). Pediatric Primary Care (5th ed.). Philadelphia: Elsevier Health.
Domino, F. (2014). The 5- Minute Clinical Consult (14th ed.). Philadelphia:
Lippincott Williams & Wilkins.
Dunphy, L. (2011). Primary Care: The art and science of Advanced practice nursing.
(3rd ed.). Philadelphia: F.A. Davis Company.
Katzung, B., Masters, S., & Trevor, A. (2012). Basic and Clinical Pharmacology (12th
ed.). New York: McGraw Hill Medical.
Larson, R. (2014). Leukemia. UpToDate. Retrieved from
http://www.uptodateonline.com.
Lee, S. (2009). Pathology 2: Leukemia chart. NCNM. Retrieved from
http://ncnmnotes.blogspot.com/2009/12/pathology-ii-leukemiachart.html.
National Cancer Institute. (2013). Childhood Acute Lymphoblastic Leukemia
Treatment. National Institute of Health. Retrieved from
http://www.cancer.gov/cancertopics/pdq/treatment/childALL/Patient/p
age1.