Chronic Myeloproliferative Disorders: CML, MF, PV, ET
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| (T or F) Myeloproliferative disorders exhibit increased production of one or more peripheral blood lineage with dysplasia. | show 🗑
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| (T or F) All myeloproliferative disorders exhibit expansion of stem cells without block in maturation. | show 🗑
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| What is the molecular defect associated with all myeloproliferative disorders? | show 🗑
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| show | (1) Fatigue (2) Weight loss (3) Abdominal pain or fullness (4) Easy bruising or bleeding
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| show | (1) Splenomegaly (2) Hepatomegaly (3) Fever, hypermetabolic state (4) Purpura
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| show | Decreased
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| What are the characteristic leukocyte uric acid levels in Chronic Myelogenous Leukemia (CML)? | show 🗑
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| show | High leukocyte alkaline phosphatase (LAP) indicates a reactive process where as CML typically has decreased LAP levels.
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| What is the pathogenesis of Chronic Myelogenous Leukemia (CML)? | show 🗑
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| What are the phases of Chronic Myelogenous Leukemia (CML)? | show 🗑
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| What type of acute leukemia does majority of Chronic Myelogenous Leukemia (CML) progress to? | show 🗑
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| show | Allogeneic bone marrow transplant
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| What is the mechanism of Gleevec? | show 🗑
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| show | Gleevec
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| show | (1) Gleevec (2) Interferon
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| What are the treatment options for Chronic Myelogenous Leukemia (CML)? | show 🗑
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| show | PV is a disease of older adults, with median age at diagnosis of 60 years.
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| show | (1) Headaches (2) Dyspnea (3) Sweats (4) Dizziness (5) Pruritis
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| show | (1) Splenomegaly (2) Hepatomegaly (3) Plethora (4) Hypertension
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| What are majority of the symtpoms in Polycythemia vera (PV) a result of? | show 🗑
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| What are the bone marrow biopsy findings of Polycythemia vera (PV)? | show 🗑
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| What are the diagnostic criteria for Polycythemia vera (PV)? | show 🗑
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| What are causes of secondary erythrocytosis? | show 🗑
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| show | JAK2 is a tyrosine kinase that is downstream of the Epo receptor. Mutant JAK2 V617F has constitutively active tyrosine kinase activity mimicking Epo signaling resulting in activation of transcription factors, stem cell expansion, megakaryopoiesis.
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| Treatment for Polycythemia vera (PV) | show 🗑
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| What level must the hematocrit be maintained in phlebotomy treatment of Polycythemia vera (PV)? | show 🗑
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| show | It is a specific inhibitor of megakaryocyte maturaiton to control thrmbocytosis.
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| What are the possible course of progressive Polycythemia vera (PV)? | show 🗑
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| show | 2%
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| show | 10%
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| show | 13%
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| What is the incidence of transformation of PV to AML with 32P treatment? | show 🗑
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| (T or F) Essential thrmbocytosis is at low risk of tranformation to leukemia or myelofibrosis. | show 🗑
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| What are the clinical features of essential thrombocytosis? | show 🗑
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| What medications can exacerbate the hemorrhagic complications of essential thrombocytosis? | show 🗑
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| show | Platelet count > 600,000 with normal hemoblogin and red cell mass
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| show | Megakryocyte heyperplasia with presence of iron in the marrow
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| What conditions are associated with reactive thrombocytosis? | show 🗑
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| What is the indications for treatment of essential thrombocytosis? | show 🗑
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| show | ASA
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| show | False. Treatment doe snot appear to decrease risk of 1st trimester miscarriages.
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| What pharmacologic agents treat essential thrombocytosis? | show 🗑
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| show | Interferon
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| What are the symptoms of Chronic Idiopathic Myelofibrosis? | show 🗑
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| show | (1) Splenomegly (2) Hepatomegaly
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| Microscopic findings of Chronic Idiopathic Myelofibrosis on peripheral blood smear | show 🗑
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| Bone marrow biopsy findings of Chronic Idiopathic Myelofibrosis | show 🗑
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| Location of extramedullary hematopoiesis in Chronic Idiopathic Myelofibrosis | show 🗑
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| Treatment for thrombocytopenia in Chronic Idiopathic Myelofibrosis | show 🗑
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| show | Trial of erythropoietin, androgens. Transfusion as needed.
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