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70 Cards in this Set
- Front
- Back
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What is the mechanism of polycythemia vera?
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Neoplastic disease from hematopoietic stem cell = over production of erythroid, myeloid, megas.
BFU-E are hypersensitive to Epo, IL-3, IGF-1 |
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What is the common mutation in polycythemia vera?
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Jak2
Note: this is also mutated in 50% of essential thrombocytosis |
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Symptoms of polycythemia vera?
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Age > 60
Headache, dizziness, pruritis, erythromelalgia (burning in fingers/toes from distal ischemia), or asymptomatic. My have facial plethora, hepatosplenomegaly. |
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How is polycythemia vera diagnosed?
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Endogenous erythroid colony assay
Elevated WBC, platelets, Hct Decreased Epo Smear: basophilia, immature WBC No need to look at marrow. |
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How is polycythemia vera treated?
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Phlebotomy
Aspirin Myelosuppression (hydroxyurea) Interferon-a (poorly tolerated) P32 DO NOT use chlorambucil due to increased risk of AML. |
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What are complications of polycythemia vera?
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Increased risk of thrombosis
and/or bleeding Can progress to AML |
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What is the mechanism of LAD?
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Congenital defect in PMN adherence: failure to express integrin CD11/CD18 complex. PMNs can’t marginate.
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How is LAD diagnosed?
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Flow cytometry identifies absence of CD11/CD18
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How is LAD treated?
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Stem cell transplant (future = gene therapy?)
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What test is used to diagnose chronic granulomatous disease?
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NBT test: dye added to stimulated PMNs. If superoxide generated, dye turns blue. Flow cytometry and genetics are also used.
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Patients with CGD are at an increased risk for infection by what kind of organisms? (Examples?)
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Catalase +
S. aureus, S. marcesans |
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What are dohle bodies?
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"Toxic granulations" made of blue RNA from retained ER fragments; seen during severe neutrophilia
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When are atypical (reactive) lymphocytes often seen?
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Viral infections (esp. EBV-mono) and CLL
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What are the "sanctuaries" in ALL?
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CNS (brain) and testes
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What is the treatment for APL?
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All Trans Retinoic Acid (ATRA)
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What confers poor prognosis to patients with AML?
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Flt3 internal tandem duplication
Preceding myelodysplastic syndrome >60yo Cytogenetic abnormalities of chromosomes 5,7,11q23, bcr-abl translocation Failure to remiss w/ 1 cycle of chemo |
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What confers good prognosis to patients with AML?
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Young age
Translocations: t(15,17), t(8,21) Inversions: inv(16) NPM1 |
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What malignancy is characterized by marrow hypercellularity with > 20% blasts?
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AML
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What is the preferred "induction" chemo regimen for AML?
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"7+3": 7 day infusion of ara-C + 3 day infusion of daunorubicin
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Name 6 myeloproliferative disorders.
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1. Polycythemia vera
2. Essential thrombocytosis 3. CML 4. Primary myelofibrosis 5. Chonic Neutrophilic Leukemia 6. Chronic Eosinophilic Leukemia/HyperEosinophilic Syndrome |
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Name 3 growth factors that stimulate stem cell growth and differentiation.
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1. SCF
2. IL-3 3. GM-CSF |
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Name 4 causes of neutrophil functional defects.
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1. LAD
2. CGD 3. Chediak-Higashi Syndrome 4. Myeloperoxidase Deficiency |
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What is a differential diagnosis for neutrophilia?
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1. Physiologic response to infection
2. Transient d/t exercise or epi 3. Inflammation 4. CML 5. Polycythemia vera 6. Essential thrombocytosis |
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What is Ham's test used to diagnose?
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PNH
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How can you tell CLL apart from infection?
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flow cytometry
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How can you tell CML apart from infection?
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LAP (leukocyte alkaline phosphatase) levels
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What do the presence of auer rods indicate?
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AML
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What causes macroovalocytes and hypersegmented PMNs?
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Vitamin B12 or folate deficiency
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Target cells are seen in what conditions?
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1. Hb C
2. Asplenia 3. Liver disease 4. Thalassemia 5. Iron Deficiency Anemia |
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What can cause schistocytes?
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1. DIC
2. TTP 3. HUS 4. Carcinomatosis 5. HELLP 6. "Waring Blender Effect" |
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How is Waldenstrom's Macroglobulinemia diagnosed?
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Large IgM band on SPEP + other clinical symptoms like mild lymphadenopathy
Serum IgM > 2.5 g/dL Pancytopenia, marrow replacement |
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What is the symptomatic triad seen in Waldenstrom's Macroglobulinemia?
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1. Bleeding
2. Visual changes 3. Neurologic changes (due to occlusion of small vessels) |
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How is multiple myeloma diagnosed?
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SPEP spike: IgG>IgA>IgM>IgD
Rouleaux present in blood smear Serum monoclonal component (Ig > 3.5 g/dL) + proteinuria |
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Treatment regimen for aggressive NHL?
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CHOP+R = 40-80% cure rate depending on stage
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In which conditon do you see Reed Sternburg cells?
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Hodgkin's Lymphoma
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Treatment regimen for Hodgkin's Lymphoma?
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ABVD > MOPP
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In which condition are smudge cells seen?
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CLL
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What condition is characterized by WBC counts of 25-300K, decreased LAP levels and Ph' translocations?
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CML
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Most common leukemia of childhood?
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ALL
80% B cell; 20% T cell |
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Most common leukemia of adulthood?
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AML
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HIV is associated with a 100-fold incrased risk of acquiring what cancer?
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Aggressive B cell lymphoma
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HTLV-1 is associated with the development of what cancer?
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Adult T-cell leukemia
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EBV is associated with the development of what cancer?
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Burkitts lymphoma
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H. pylori is associated with the development of what cancer?
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gastric B cell lymphoma
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In what hematological disorders might you see basophilia?
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1. Polycythemia vera
2. CML |
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Lymphoma Stage I
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One LN region or extralymphatic site
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Lymphoma Stage II
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≥ 2 sites on same side of diaphragm
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Lymphoma Stage III
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Sites above AND below diaphragm
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Lymphoma Stage IV
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Disseminated involvement of a visceral organ (ex: bone marrow or liver)
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What are some complications of treating Hodgkin's Lymphoma?
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1. Secondary malignancies in up to 20% in 20 years (AML, NHL, Solid tumors)
2. Hypothyroidism 3. Sterility (esp. MOPP) 4. Adriamycin Cardiomyopathy 5. Bleomycin Lung 6. Premature CAD |
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Indications for treating multiple myeloma.
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"CRAB":
hyperCalcemia Renal failure Anemia Bone lesions (punched-out) |
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Inherited causes of thrombophilia?
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Factor V Leiden
Prothrombin Antithrombin III Deficiency Protein C Deficiency Protein S Deficiency Others |
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Acquired causes of thrombophilia?
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Antiphospholipid antibody (Lupus anticoagulant)
Myeloproliferative disorder Malignancy Heparin induced Thrombocytopenia PNH Estrogen Pregnancy |
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What hematological test is prolonged in factor VII deficiency?
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PT
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What factor deficiencies result in a prolonged PTT?
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Factor VIII
Factor IX Factor XI |
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What lab findings would you see in someone with Lupus Anticoagulant?
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Prolonged PTT and NO correction on 1:1 mixing
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What lab findings would you see in someone with antibodies to Factors VIII or IX? How you would tell this apart from hemophilia?
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Prolonged PTT
1:1 mixing will NOT be corrected with antibodies to factors VIII and IX but will correct in hemophilias |
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What is the earliest recognizable myeloid cell in the bone marrow?
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Myeloblast
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CD19 is present on what kind of cells?
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B cells
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CD34 and CD15 are present on what kind of cells?
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Myeloid
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What is the translocation and fusion gene associated with CML?
Specific therapy? |
t(9,22)
bcr-abl Imatinib (Gleevec) |
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What is the translocation and fusion gene associated with APL?
Specific therapy? |
t(15,17)
RAR-alpha/PML All Trans Retinoic Acid (ATRA) |
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What is the translocation and fusion gene associated with follicular NHL?
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t(14,18)
IgH-bcl2 |
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What chemo agent is used for AML and ALL
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High dose ara-C
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Rituximab mechansm? Indicaitons?
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CD20 antibody; used mainly for NHL but can be added to other treatment regimens
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Indications for fludarabine?
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CLL
Waldenstrom’s NHL AML mini-transplant |
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Indications for HyperCVAD?
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ALL
Aggressive NHL |
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Hodgkin's disease classification?
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Classical HD:
Nodular Sclerosing Mixed Cellularity Lymphocyte Depleted Lymphocyte Rich Classical HD Nodular lymphocyte predominant HD (really a B-cell NHL) |
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Characteristics of MM?
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1. Bone marrow plasmacytosis (usually >30%)
2. Monoclonal gammopathy (“M protein”) 3. Lytic Bone Lesions 4. Plasmacytomas |
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What is the goal of performing an autologous bone marrow transplant on an MM patient?
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Improve survival (not curative)
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