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67 Cards in this Set

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Define Leukemia
Primary neoplasia of the Bone Marrow
-usually goes into the blood
Define Lymphoma
Primary neoplasm of the Lymph Node or of Extra-nodal Lymphoid tissue
-GI tract, Thyroid, Salivary gland, skin

**RARELY goes into the Blood
Defie "Acute Leukemia"
1. Proliferation of IMMATURE cells

2. Survival 30 days without therapy

**poorly differentiated hematopoietic cells called "Blast cells"
Define "Chronic Leukemia"
1. Proliferation of MATURE cells

2. Survival several years without therapy

**well-differentiated hematopoietic cells
Bone Marrow neoplasm with proliferation of IMMATURE MYELOID stem cells (Myeloblasts) that involves the blood 99% of the time
Acute Myelogenous Leukemia
Acute Myelogenous Leukemia Epidemiology:
1. Age

2. Gender preference
1. Adults: Median = 40-50

2. Males > Females

AMl = Adult Males
What environmental factors can predispose to AML?
1. Radiation = pts who get radiated for other tumors are at risk

2. Drugs
- DNA topoisomerase inhibitors = Etoposide
- Alkylating agents = Cyclophosphamide
What diseases are risk factors for AML? (4)
1. Myelodysplastic syndrome --> 10-40% risk of AML
2. Paroxysmal Nocturnal Hemoglobinuria
3. CML
4. Down Syndrome
Other than Environmental factors and diseases, what other factor plays a role in the pathogenesis of Acute Myelogenous Leukemia?
Genetic mutations involving translocations
What is seen in the blood of Acute Myelogenous Leukemia patients?
1. Pancytopenia
-Anemia = fatigue, weakness
-Thrombocytopenia = bleeding, petechial skin rash
-Leukopenia = recurrent infections

2. Myeloblasts
What are the pathological findings in the Bone Marrow of Acute Myelogenous Leukemia patients? (3)
1. Bone Marrow Blasts > 20%
2. Cytoplasmic Auer rods
3. Myeloperoxidase or Sudan Black B positivity
Auer rods = what disease?
Acute Myelogenous Leukemia

**aggregated Primary Granules
AML Classification:
1. M0 =
2. M1 =
3. M2 =
4. M3 =
5. M4 =
6. M5 =
7. M6 =
8. M7 =
M0: Minimally differentiated
M1: Myeloblastic leukemia w/out maturation
M2: Myeloblastic Leukemia w/ maturation = some Promyelocytes = most common type
M3: Promyelocyte; t(15;17
M4: Myelomonocytic = Myeloblasts and Monoblasts
M5: Monocytic
M6: Erythrocytic
M7: Megakaryocytic
What are the clinical features of AML?
1. Weakness = anemia
2. Bleeding = Thrombocytopenia
3. Infection = neutropenia
4. Hepatosplenomegaly
What AML is associated with DIC? Why?
M3 = Promyelocytic = release of thromboplastic substances in granules
What are type M4 & M5 AML associated with?
Skin, Gingival, and Perianal infiltration of Monocytes
What is the 5 year survival rate for Acute Myelogenous Leukemia?
15-30%
What are the 2 favorable features to have if a patient has Acute Myelogenous Leukemia?
1. t(8;21) = M2

2. Inversion 16 = M4
What are 3 unfavorable features to have if you are diagnosed with AML?
1. t(9;22) = Philadelphia chromosome

2. 11q23 translocations = seen in M4 & M5

3. Chromosome 5 or 7 deletions = seen in Myelodysplasia
What is the treatment for favorable cases of AML? Unfavorable?
Favorable = Multiagent Chemotherapy

Unfavorable = BM transplant
What treatment is used for M3 AMLs? Why?
Trans-retinoic acid = reduces the risk of DIC by inducing Promyelocytes to mature = loss of primary granules

**t(15;17)
-15 = Polymorphonuclear Leukocyte gene (PML)
-17 = Retinoic Acid Receptor alpha gene
-forms abnormal retinoic acid receptor
This is a group of clonal hematopoietic stem cell neoplasms that results in either:
1. excessive release of mature myeloid cells into blood
OR
2. Bone Marrow fibrosis
Chronic Myeloproliferative Disorders
What are the 4 disorders that comprise Chronic Myeloproliferative Disorders
1. Chronic Myelogenous Leukemia
2. Polycythemia vera
3. Essential Thrombocytosis
4. Primary Myelofibrosis
What are the common features of Chronic Myeloproliferative Disroders?
1. Middle aged to elderly
2. Splenomegaly
3. Bone Marrow Panhypercellularity
-Neutrophil precursors
-Erythroid precursors
-Megakaryocytes
Factors contributing to CML:
1. Environmental (2)

2. Genetic (2)
1. Radiation & Benzene

2. HLA-Cw3 & Cw4
Philadelphia Chromosome = t(9;22)

What disease?
Chronic Myelogenous Leukemia
Describe the Philadelphia chromosome
t(9;22) translocation
-9 = c-abl oncogene
-22 = break cluster region (bcr)

**results in constitutive activity of Tyrosine Kinase = promotes cell proliferation and inhibits Apoptosis
What pathologies are seen in the blood of CML patients?
1. Marked Neutrophilia (>50,000 / uL)
2. Entire spectrum of Neutrophil maturation
-this is not seen in Reactive Neutrophilia
3. Basophilia
4. decreased Leukocyte Alkaline Phosphatase (LAP)

**No toxic granulation or Dohle bodies are seen in CML; these are seen in Reactive Neutrophilia due to infection
What are the clinical features of CML?
1. Fatigue and Weight loss = hypermetabolic state
2. Early satiety and LUQ pain = due to Splenomegaly
3. Bleeding and Thrombosis = due to irregular platelets
What is the survival rate for CML without therapy?
Median is 3-4 years
What are the 2 possible complications of CML?
1. Transformation into ACUTE Leukemia = Blast Crisis
- AML = 70%
- ALL = 30%

2. Bone Marrow Failure
What are the 3 treatment options for CML?
1. Gleevec = tyrosine kinase inhibitor = only controls disease, doesn't cure

2. Alpha IFN

3. BM transplantation
What is the Leukemoid Reactions?
Markedly elevated blood neutrophil count > 50,000/uL with all stages of neutrophil maturation, due to a REACTIVE cause (usually severe sepsis)
- mimicks CML
How would you tell the difference between CML and Leukemoid Reaction?
Leukemoid rxns will NOT have Splenomegaly or Basophils; Philadelphia Chromosome will be absent
Describe Polycythemia Vera
Unknown cause of an Autonomous Erythroid proliferation that is independent of EPO

**increased # of RBC's not due to increased EPO
What blood pathologies are seen in Polycythemia Vera?
1. Elevated Hemoglobin
2. Elevated Hematocrit
3. Increased Plasma volume
4. Elevated RBC mass = diagnostic
5. Decreased serum EPO = feedback inhibition due to RBC count
What pathology is seen in the Bone Marrow in Polycythemia vera?
Panhypercellular
What are the clinical features of Polycythemia vera?
1. Plethora = excess of blood
2. Hyperviscosity syndrome
-Headache, dizziness, visual disturbances = due to slowed blood flow through the brain
3. Thrombosis = due to increased viscosity
4. HTN
5. Pruritis = due to histamine release from Basophils
5. Peptic Ulcers = due to histamine release from Basophils
6. Splenomegaly
54-year old man presents with headache and blurry vision. PMI includes HTN of 10 years duration and 2 recent hospital admissions for deep vein thrombosis. Blood tests reveal increased hematocrit and RBC mass.
Polycythemia vera
-Headache/blurry vision = Hyperviscosity syndrome
-HTN
-Thrombosis = due to increased viscosity
What is the survival rate for Polycythemia vera?
Median survival is > 10 years
What can be a complication of Polycythemia vera?
Transformation to AML (5-10% of cases)
What are the treatments available for Polycythemia vera? (3)
1. Phlebotomy
2. Hydroxyurea = only used if RBC production is too fast and phlebotomy doesn't work
3. Busulfan = increases the risk of AML so not as widely used
What is the pathology of Essential Thrombocytosis?
1. Elevated platelets > 600K/uL
2. Giant platelets
3. Bone Marrow panhypercellularity
Giant platelets
Essential Thrombocytosis
What are the 3 clinical features of Essential Thrombocythemia
1. Bleeding
2. Thrombosis
3. Splenomegaly

**Platelets are dysfunctional and can go in any direction = Bleeding or Thrombosis
What is the prognosis for Essential Thrombocytosis?
Median survival > 10 years
What can be a complication of Essential Thrombocytosis?
Transformation to AML
What are 3 treatments for Essential Thrombocytosis?
1st line = Hydroxyurea
2nd line = Anegrelide
3rd line = Alpha interferon
Describe the pathogenesis of Primary Myelofibrosis
Neoplasm of Megakaryocytes produce excess amounts of PDGF and TGF-beta, which stimulate Fibroblasts and leads to BM fibrosis
What pathologies are seen in the Blood in Primary Myelofibrosis
1. Pancytopenia
2. Tear drop red cells
3. Leukoerythroblastic reaction = immature neutrophils and nucleated red cells in peripheral blood
Tear drop red cells
Primary Myelofibrosis
Leukoerythroblastic reaction is seen in this disease
Primary Myelofibrosis
What pathologies are seen in the Bone Marrow in Primary Myelofibrosis?
Fibrosis

Osteosclerosis
What are the clinical features of Primary Myelofibrosis?
1. Fatigue, bleeding, infection = Pancytopenia
2. Weight loss
3. Gout = due to high cell turnover which causes hyperuricemia
4. Splenomegaly = due to extramedullary hematopoiesis = myeloid metaplasia
What is the prognosis of Primary Myelofibrosis?
Median survival is 3-4 years
What is the complication of Primary Myelofibrosis?
AML transformation in 15-20% of patients
What is the treatment for Primary Myelofibrosis?
Supportive
-transfusions
-antibiotics
Auer rods = aggregated primary granules in promyelocytes

Acute Myelogenous Leukemia subtype M3 (can be seen in any AML)
What are these called?

What disease?
This is a blood smear from what disease? How do you know?
Chronic Myelogenous Leukemia
-Neutrophils at all stages of maturation
-Basophilia
Chronic Myelogenous Leukemia
-no Fat cells
-granulocytic and megakaryocytic hyperplasia = panhypercellular
This is a Bone Marrow sample from what disease?
Giant Platelet = platelets are normally 1/3 to 1/2 the size of RBC's

Essential Thrombocytosis
What is the arrow pointing at?

What disease?
Primary Myelofibrosis
-Tear-drop cells
-Nucleated Red Cells
What disease is this? How do you know?
Bone Marrow of Primary Myelofibrosis
-bony trabeculae that are very thickened
-Eosinophilic material in the medullary cavity = Fibrosis
-Dominated by fibrosis and Osteosclerosis
What disease is this?
A 44-year-old man presents complaining of fatigue and night sweats. Physical examination is unremarkable except for an enlarged spleen. Lab findings reveal a high WBC counts, multiple granulocytes of all levels of development on peripheral blood smear, and low leukocyte alkaline phosphatase activity (LAP)
Chronic Myelogenous Leukemia
Decreased Hematocrit with Teardrop-shaped RBC's
Primary Myelofibrosis
Thrombocytosis in the peripheral blood and Megakaryocytosis in the Bone Marrow

Patient has both bleeding and Thrombosis events
Essential Thrombocythemia
-Increased Hematocrit
-Increased RBC mass
-Decreased Erythropoietin
Polycythemia Vera