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14 Cards in this Set
- Front
- Back
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What dz is JAK2 mutation found in?
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PV, ET, CIMF
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What does JAK mutation mean?
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poor prognosis
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What does philadelphia chromosome presence indicate?
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poor prognosis in anything other than CML
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What causes hepatosplenomegaly in myeloproliferative disorders?
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extramedullary hematopoeisis and sequestration of extra cells
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What proteins are increased in CML? Effect?
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myc and bcl-2 are anti-apoptotic
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Clinical course of CML?
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indolent phase followed by one or both of accelerated/blast (bi- or tri-phasic)
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Txs for CML?
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1)gleevec 2ndline-other tk inhibitors 3rdline or blast crisis-BMT
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Course of polycythemia vera?
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10% progresses to spent phase, 10% to myelofibrosis, 10%to AML
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Tx for PV?
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phlebotomy, hydroxyurea (decreases risk for thrombosis)
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Tx for ET?
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plateletpheresis, hydroxyurea, anagrelide (reduces megakar size)
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What causes CIMF?
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growth factors produced by abn hematopoeitic cells (especially megas)
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What is the pathopys of CIMF?
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reticulin fibers initially…collagen later
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What kills you in CIMF?
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thrombosis, infxn, hemorrhage, portal htn, cardiac failure, aml
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When is splenectomy indicated in CIMF?
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painful slenomegaly, too much transfusion required, hemanem, severe thrombocytopenia, portal htn
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