Term
| What are 4 major Myeloproliferative Neoplasms defined as? |
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Definition
| Chronic Myelogenous Leukemia, Polycthaemia vera, Primary Myelofibrosis, Essential Thrombocythaemia |
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Term
| What is the Myeloproliferative neoplasm associated with t9:22? |
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Definition
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Term
| What are the three Myeloproliferative Neoplasms associated with Jak2? |
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Definition
| Polycythemia Vera (PV), Essential Thrombocythemia (ET), and Primary Myelofibrosis (PMF) |
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Term
| What proliferates in Primary Myelofibrosis? |
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Definition
| Megakaryocytes and Granulocytes (NOT erythroid precursors) |
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Term
| What happens to the marrow over time with primary Myelofibrosis? |
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Definition
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Term
| What should you expect to see during the Prefibrotic and early stage of primary Myelofibrosis? |
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Definition
| Mild left shift, Hypercellular marrow, Bizzare Megakaryocytes (pleomorphic and clustered) |
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Term
| What should you expect to see during the Fibrotic stage of primary Myelofibrosis? |
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Definition
| Myelophthisic smear (Tear drop RBCs and nucleated RBCs), Fibrosis, Extramedullary hematopoiesis |
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Term
| Most cases of primary Myelofibrosis are due to what mutations? |
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Definition
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Term
| What is the overall survival of Primary Myelofibrosis if diagnosed in fibrotic stage and what can it progress to? |
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Definition
| 3-7 years; Can progress to AML |
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Term
| Describe Polycythemia Vera |
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Definition
| Panmyelosis: (everything increased) with clonal MPN characterized by proliferation of erythroid precursors independent of normal erythropoiesis control mechanisms |
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Term
| What are the three phases of Polycythemia Vera? |
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Definition
| Prodromal/Pre-polycythemic > Overt Polycythemic > Spen/Post-Polycythemic |
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Term
| What are the two different classificaitons of Polycythemia Vera? |
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Definition
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Term
| What are the two categories of Secondary Polycythemia Vera and briefly describe? |
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Definition
| Congenital (some sort of mutation) and Acquired (Physiological response like increased EPO due to hypoxia) |
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Term
| What causes Spurious Polycythemia Vera? |
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Definition
| Acute transient hemoconcentration due to dehydration or other causes, not really a RBC mass increase |
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Term
| When looking at Jak2 mutations and serum EPO (erythropoietin) levels, what indicates Polycythemia Vera? |
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Definition
| Jak2 mutation with EPO decrease |
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Term
| What are the clinical Symptoms of Polycythemia Vera? |
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Definition
| Hypertension, thrombosis, headache, dizzy, PRURITUS, Splenomegaly |
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Term
| What mutation is present in almost all (>95%) Polycythemia Vera cases? |
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Definition
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Term
| What is the overall survival and cause of death in Polycythemia Vera? |
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Definition
| >10 year survival; Death from Thrombosis or hemorrhage |
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Term
| Describe Essential Thrombocythemia |
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Definition
| Clonal MPN characterized by sustained thrombocytosis with increased large marrow Megakaryocytes (not really clumped together but largely increased) |
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Term
| What type of appearance do the megakaryocytes take on in Essential Thrombocythemia? |
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Definition
| Stag-horn appearance (large but not bizarre) |
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Term
| What is the clinical presentation of Essential Thrombocythemia? |
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Definition
| Often asymptomatic, splenomegaly is rare and no fibrosis occurs |
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Term
| What mutation is positive in 50% of Essential Thrombocythemia cases? |
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Definition
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Term
| What is the prognosis of Essential Thrombocythemia? |
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Definition
| Good, mostly and indolent disease |
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Term
| What is the clinical appearance of CML? |
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Definition
| Fatigue, Splenomegaly, but mostly asymptomatic |
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Term
| What is seen on blood smear of CML? |
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Definition
| Leukocytosis with left shift, Eosinophilia and Basophilia, Myelocyte buldge, Pseudo-Gaucher cells, and Dwarf Megakaryocytes |
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Term
| What is it important to distinguish CML from and how do you do this? |
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Definition
| A leukmoid process (some infection or something); t(9:22) detection OR Leukocyte Alkaline Phosphatase (LAP) score, which will be HIGH in a leukmoid reaction |
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Term
| What are the three phases of CML? |
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Definition
| 1) Chronic, 2) Accelerated, 3) Blast (acute leukemia) |
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Term
| What defines the Accelerated phase of CML? |
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Definition
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Term
| What defines the Blast Crisis in CML? |
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Definition
| >20% blasts (doesn't matter if lymphoid or myeloid blasts) |
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Term
| What is the survival of CML in Chronic phase, Accelerated phase, and Blastic phase? |
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Definition
| Chronic: 4-6 years - Accelerated: 1 year - Blastic: 3-6 months |
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Term
| What happens to Iron stores in Polycythemia Vera? |
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Definition
| Decrease due to the overactive growth of RBCs |
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