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. | False. All myeloproliferative disorders exhibit increased production of one or more peripheral blood lineages without dysplasia.
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| (T or F) All myeloproliferative disorders exhibit expansion of stem cells without block in maturation. | True.
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| What is the molecular defect associated with all myeloproliferative disorders? | Persistent activation of tyrosine kinases
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| What are the symptoms of Chronic Myelogenous Leukemia (CML)? | (1) Fatigue (2) Weight loss (3) Abdominal pain or fullness (4) Easy bruising or bleeding
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| What are the physical signs of Chronic Myelogenous Leukemia (CML)? | (1) Splenomegaly (2) Hepatomegaly (3) Fever, hypermetabolic state (4) Purpura
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| What are the characteristic leukocyte alkaline phosphatase levels in Chronic Myelogenous Leukemia (CML)? | Decreased
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| What are the characteristic leukocyte uric acid levels in Chronic Myelogenous Leukemia (CML)? | Increased from increased cell turnover
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| How is a reactive leukocytosis from infection distinguished from a leukocytosis in Chronic Myelogenous Leukemia (CML)? | 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)? | Translocation of chromosome 9 and 22 moves bcr gene next to abl tryosine kinase gene, creating a fusion protein. Bcr-abl fusion protein results in a constitutive cytoplasmic tyrosine kianse activity, promoting cell proliferation and survival.
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| What are the phases of Chronic Myelogenous Leukemia (CML)? | (1) Chronic phase (2) Accelerated phase (3) Blast phase
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| What type of acute leukemia does majority of Chronic Myelogenous Leukemia (CML) progress to? | 85% of CML progresses to AML.
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| What is a possible curative option for Chronic Myelogenous Leukemia (CML)? | Allogeneic bone marrow transplant
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| What is the mechanism of Gleevec? | Gleevac is a Imatinib mesylate which is an inhibitor of bcr-abl tryosine kinase.
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| What is the initial treatment of Chronic Myelogenous Leukemia (CML)? | Gleevec
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| What pharmacologic agents can be used to induce remission of Chronic Myelogenous Leukemia (CML)? | (1) Gleevec (2) Interferon
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| What are the treatment options for Chronic Myelogenous Leukemia (CML)? | (1) Bone marrow transplant (2) Gleevec (3) Interferon (4) Chemotherapy
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| What is the median age of diagnosis of Polycythemia vera (PV)? | PV is a disease of older adults, with median age at diagnosis of 60 years.
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| What are the typical symptoms of Polycythemia vera (PV)? | (1) Headaches (2) Dyspnea (3) Sweats (4) Dizziness (5) Pruritis
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| What are the typical physical signs of Polycythemia vera (PV)? | (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? | Symptoms experienced are often the result of increased viscosity due to the increased hematocrit.
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| What are the bone marrow biopsy findings of Polycythemia vera (PV)? | (1) Hypercellular with erythroid hyperplasia (2) Decreased iron stores (3) Increased megakaryocytes
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| What are the diagnostic criteria for Polycythemia vera (PV)? | Presence of 4 of the following: (1)Elevated red cell mass (2)Normal arterial oxygen saturation (3)Thrmbocytosis and leukocytosis (4)Marrow hypercelluarity with absent iron stores (5)Low erythropoietin level (6)Abnormal marrow proliferative capacity
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| What are causes of secondary erythrocytosis? | (1) Hemoconcentration (2) Hypoxia (3) CO intoxification (4) Inappropriate erythropoietin production
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| Pathophysiology of Polycythemia vera (PV) | 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) | (1) Phlebotomy (2) Hydroxyurea (3) Anagrelide (4) ASA or NSAID (5) others: 32P, busulfan, IFN-alpha, allopurinol, muatant JAK2 inhibitor
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| What level must the hematocrit be maintained in phlebotomy treatment of Polycythemia vera (PV)? | Hct < 45%
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| What is the mechanism of Anagrelide? | 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)? | (1) Spent phase: post-polycythemic myelofibrosis (2) Transformation to AML
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| What is the incidence of transformation of PV to AML with phlebotomy treatment? | 2%
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| What is the incidence of transformation of PV to AML with hydroxyurea treatment? | 10%
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| What is the incidence of transformation of PV to AML with chlorambucil treatment? | 13%
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| What is the incidence of transformation of PV to AML with 32P treatment? | 10%
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| (T or F) Essential thrmbocytosis is at low risk of tranformation to leukemia or myelofibrosis. | True.
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| What are the clinical features of essential thrombocytosis? | Thrombotic complications (headaches, visual disturbances, erythromelalgia) (2) Hemorrhagic complications (superifical cutaneous or mucosal bleeding) (3) splenomegaly
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| What medications can exacerbate the hemorrhagic complications of essential thrombocytosis? | ASA or NSAIDs
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| What is the typical CBC of essential thrombocytosis? | Platelet count > 600,000 with normal hemoblogin and red cell mass
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| Bone marrow biopsy findings of essential thrmbocytosis | Megakryocyte heyperplasia with presence of iron in the marrow
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| What conditions are associated with reactive thrombocytosis? | (1) Infectious/inflammatory states (2) Surgical procedures or tissue damage (3) Malignancy (4) Fe deficiency, hemolytic anemia, acute blood loss (5) Post-splenectomy (6) Rebound post chemotherapy or ITP (7) Renal failure/nephrotic syndrome.
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| What is the indications for treatment of essential thrombocytosis? | (1) symptomatic (2) age>60 (3) history of thrombosis (4) cardiovascular risk factors
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| What pharmacologic agents are used to treat vasomotor symptoms of essential thrombocytosis? | ASA
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| (T or F) Treatment decreases the risk of miscarriage in the 1st trimester in essential thrombocytosis. | False. Treatment doe snot appear to decrease risk of 1st trimester miscarriages.
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| What pharmacologic agents treat essential thrombocytosis? | (1) Hydroxyurea (2) Angrelide (3) Interferon
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| What pharmacologic agent can be used to treat essential thrombocytosis in pregnant patients? | Interferon
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| What are the symptoms of Chronic Idiopathic Myelofibrosis? | (1) Fatigue (2) Weight loss (3) Night sweats
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| What are the physical signs of Chronic Idiopathic Myelofibrosis? | (1) Splenomegly (2) Hepatomegaly
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| Microscopic findings of Chronic Idiopathic Myelofibrosis on peripheral blood smear | Leukoerythroblastic blood smear: (1) Immature meyloid cells (2) Nucleated RBC (3) Teardrop cells
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| Bone marrow biopsy findings of Chronic Idiopathic Myelofibrosis | (1) Extensive marrow fibrosis (2) Dysplastic megakaryocyte hyperplasia (3) Dilation of marrow siusoids with intravascular hematopoiesis (4) Osteosclerosis in advanced disease
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| Location of extramedullary hematopoiesis in Chronic Idiopathic Myelofibrosis | (1) spleen (2) liver
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| Treatment for thrombocytopenia in Chronic Idiopathic Myelofibrosis | Decrease spleen size: (1) chemotherapy (2) Radiation (3) Splenectomy
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| Treatment for anemia in Chronic Idiopathic Myelofibrosis | Trial of erythropoietin, androgens. Transfusion as needed.
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