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14 Cards in this Set

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