Rita All Carrie Warner Definition 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 ID: 330909
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Slide1
Leukemia
Rita All
Carrie WarnerSlide2
Definition
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).Slide3
Normal blood versus leukemia blood
(American Cancer Society, 2014)Slide4
Leukemia Vs. Lymphoma
According to the American Joint Committee on Cancer (AJCC)
Circulating cells= leukemia
Tumor masses in lymph nodes and tissues= lymphoma
If both= lymphoma/leukemia
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)Slide5
Pathophysiology
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)Slide6
Genesis of blood cells
(National Cancer Institute, 2013)Slide7
Etiology
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). Slide8
Incidence
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)Slide9
Leukemias
(Lee, 2009)Slide10
Screening
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
organomegaly
-
hepatosplenomegaly
, enlarged lymph nodes.
CBC with Differential (Often found during routine exam or illness)
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)Slide11
Risk Factors
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).Slide12
Clinical Findings
Children:
Signs and symptoms related to leukemic replacement of bone marrow/ absence of blood cell precursors
Anemia, Pallor, listlessness
Irritability, chronically tired
History of repeated infections, fever, weight loss
Bleeding-
epistaxis
,
petechiae
, hematomas
Lymphadenopathy
and
hepatosplenomegaly
Bone and joint pain
CNS symptoms if brain involvement
Abnormal CBC, low or high white cell count with out of proportion differential
Testicular pain
(Burns, 2013)
Adults:
Acute forms have bone and joint pain
Gingival hyperplasia with bleeding
S/S infection- Fevers, chills, palpitations, shortness of breath
Skin eruptions, easy bruising, prolonged bleeding times
Chronic forms c/o fatigue, night sweats, low grade fevers
CML-
leukostasis
- blurred vision, respiratory distress,
priapism
Organomegaly
with chronic form,
hepatosplenomegaly
with enlarged spleen and lymph nodes, assoc with N/V
Pallor, Anemia
Abnormal CBC, low or high white cell count with out of proportion differential
(
Dunphy
, 2011)Slide13
Differential Diagnosis
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
Aplastic
anemia
Myelofibrosis
Autoimmune disease (lupus,
felty
syndrome)
Infectious mononucleosis
Pertussis
Autoimmune Thrombocytopenia
purpura
Leukemoid
reaction to infection
(Domino, 2014).
Acute Myeloid Leukemia
Virus Induced
Cytopenia
,
lymphadenopathy
, and
organomegaly
Immune
Cytopenias
(including Systemic lupus
erythmatosis
)
Drug induced
cytopenias
Other marrow failure and infiltrative disease (
aplastic
anemia, paroxysmal nocturnal
hemoglobinuria
,
myelodysplastic
syndromes,
Gaucher
disease)
(Domino, 2014).Slide14
Differential Diagnosis
Chronic
Myelogenous
Leukemia
Chronic
Myelomonocytic
leukemia
Chronic
neutrophilic
leukemia
Chronic
eosinophilic
leukemia
Juvenile
myelomonocytic
leukemia
Infectious mononucleosis
Leukemoid
reaction
Polycythemia
vera
Treatment with granulocyte stimulating factors
Acute
myelogenous
leukemia
Acute lymphoblastic leukemia
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
).Slide15
Social/ Environmental Considerations
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.
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)Slide16
Laboratory tests/ diagnostics
CBC with Manual Differential
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
Uric acid level
Can be elevated or high
LDH
Elevated
PCR (polymerase chain reaction)
Presence of biomarkers in blood/ bone marrow cells . DNA abnormality marker with AML or CML “
philadelphia
chromosome”
Liver Function Tests
Elevation due to inflammation of liver
Sed
Rate
Elevation
CRP
Elevation
Bone marrow
Bx
/ aspiration
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)Slide17
Management/ Treatment Guidelines
Treatment varies on
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). Slide18
Management/ Treatment Guidelines
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)Slide19
Treatment guidelines
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 antibodies-
rituximab
,
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).Slide20
Leukemia pharmacology
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.
(
Katzung
, Masters, & Trevor, 2012).Slide21
Complications
Infection
Anemia
Excess bleeding/ bruising (low PLT)
Clotting disorders ( elevated PLT), DVT, CVA
Richter transformation- 3-5% of patients with CLL develop diffuse large B cell lymphoma, and prognosis is generally poor.
Other cancer developments due to cancer or treatments- Kaposi Sarcoma, Melanoma, Bladder, Lung, stomach, throat cancers.
Hairy cell leukemia- can develop
hodgkins
, non-
hodgkins
, thyroid ca.
Kidney failure (Rare)
Ruptured spleen (due to enlargement)
CNS involvement- seizures, headache, vomiting, confusion, loss of coordination
Depression
Pain (increased WBC in marrow)
Infertility
Death
Therapy related illness, including
avascular
necrosis, transfusion reaction, neurotoxicity, tumor
lysis
syndrome,
Late effects (66%)- learning disabilities, poor work performance, psyche distress, health insurance discrimination, “chemo brain”- inability to concentrate.
(
Dunphy
, 2011; Burns, 2013)Slide22
Follow up
Chronic
leukemias
Recommended check ups every 6 months
Acute
leukemias
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). Slide23
Counseling/ Education
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). Slide24
Consultation/ Referral
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
Complementary therapists: Acupuncture, Reiki, Meditation, Hypnosis,
Aromatherapist
Hospice
Pain management
Cancer rehabilitation
Home care
Psychiatrist, counselors
Physical therapy
Spiritual/ Religious counselors
(National Cancer Institute, 2013). Slide25
Questions
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.) CLLSlide26
Questions
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.) ALL and CLL
b.) AML and CML
c.) ALL and AML
d.) 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.Slide27
Questions
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 historySlide28
References
American Cancer Society. (2014). Leukemia – Acute Lymphocytic (Adults). Retrieved from http://www.cancer.org/acs/groups/cid/documents/webcontent/003109-pdf.pdf.
Burns, C. (2013).
Pediatric Primary Care
(5
th
ed.). Philadelphia: Elsevier Health.
Domino, F. (2014).
The 5- Minute Clinical Consult
(14
th
ed.). Philadelphia: Lippincott Williams & Wilkins.
Dunphy
, L. (2011).
Primary Care: The art and science of Advanced practice nursing
. (3
rd
ed.). Philadelphia: F.A. Davis Company.
Katzung
, B., Masters, S., & Trevor, A. (2012).
Basic and Clinical Pharmacology
(12
th
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-leukemia-chart.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/page1.