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14 Cards in this Set
- Front
- Back
4 types of CMPDs.
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CML - Chronic Myeloblastic Leukemia
PV - Polycythemia Vera ET - Essential Thrombocythemia IMF - Idiopathic Myelofibrosis |
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General characteristics of CMPDs
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Hypercellular b.m.
Increased quantities of some or more cell lines 60-90% pts have splenomegaly Clonal |
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Clinical and hematological features of CMPDs
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middle age and older groups
extra-meduallary hematopoiesis -> spleen and liver may terminate in Acute Leukemia Frequent transition between CMPDs Cytogenic abN Molecular abN |
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IMF characteristics
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fibrosis of the marrow
extra-meduallary hematopoiesis or myeloid metaplasia leukoerythroblastosis dacryocytes present No cure |
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IMF pathogenesis & current theory
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increased acculmulation of marrow collagen
plts are implicated calcitrol |
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calcitrol
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inhibits collagen synthesis by suppressing megakaryocyte proliferation
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PV laboratory findings
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^^ rbcs in pb and bm + excessive proliferation of myeloid & plt elements
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PV pathogenesis
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- origin of PV is an abN pluripotent stem cell
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PV lab features
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1. ^rbc/^hct/^hb
2. EPO N or decrease 3. rbcs are N/N 4. ^plts (50%)/^granulocytes(68%) 5. falsely elevated PT and APTT tests 6. ^LAP (70%) 7. splenomegaly is present |
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PV risks
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1. in vivo clotting
2. transformation into IMF or leukemia |
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PV treatment
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to reduce rbc mass
- phlebotomy - myelosuppressive drugs |
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ET characteristics
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1. marked thrombocytosis
2. plts have abN plt function 3. quite rare 4. 2/3 pt no sym, 1/3 bleeding sym |
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ET lab features
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1. plts >600 x 10^9/L
2. PDW ^ (usually <20) 3. abN plt aggregation pattern 4. prolonged bleeding times 5. megakaryocytic hyperplasia in bm 6. ph chromosome is absent |
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ET treatment
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1. plateletpheresis
2. chemo |