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32 Cards in this Set
- Front
- Back
4 myeloproliferative disorders
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polycythemia vera (PV)
essential thrombocythemia (ET) idiopathic myelofibrosis (IMF) chronic myeloid leukemia (CML) |
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CML is caused by proliferation of ___ which gives rise to ___ (4).
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CFU_GEMM
BFU_E CFU_Meg CFU_GMEo CFU_baso |
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CML is caused by ___;___ translocation, aka ___.
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9
22 philadelphia chromosome |
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CML presents with ___cytosis and ___philia.
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granulo
baso |
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granulocytes in CML are mature/immature
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immature
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CML presents with thrombo___.
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cytosis
|
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CML is ___% of all leukemia with median age of diagnosis ___.
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15
55 |
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CML is linked with exposure to ___.
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radiation
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t(9;22) causes ___.
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Bcr/Abl chimera
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the Y kinase gene in the chimera is ___
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Abl
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t(9;22) effects include increased ___, altered ___, impaired ___, disrupted ___, and resistance to ___.
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proliferation
cytoadhesion maturation cell cycle regulation apoptosis |
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secondary mutations in CML can trigger ___ (2) phases
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accelerated
blast |
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6 secondary mutations in CML
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monosomy 7
trisomy 8 trisomy 9 monosomy/trisomy/isochromosome 17 |
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CML patients are usually diagnosed in ___ phase
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chronic
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despite thrombocytosis, CML patients are prone to both ___ and ___ due to ___.
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hemorrhage
thrombosis platelet dysfunction |
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hyperleukocytosis in CML can cause ___ but is often asymptomatic
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priapism
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3 physical exam findings in CML
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splenomegaly
hepatomegaly purpura |
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as thrombocytosis in CML worsens, risk shifts to ___ and away from ___.
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hemorrhage
thrombosis |
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3 lab findings in CML
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high LDH
high uric acid low/zero LAP |
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5 causes of leukocytosis
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steroids
inflammation leukemoid reaction severe infection CML |
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___ can distinguish CML from ___ (2)
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LAP score
MPD leukemoid rxn |
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___ is used clinically to identify philadelphia chromosome
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RT-PCR
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blast crisis is defined by ___
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>20% blasts in PB or BM
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chronic phase of CML typically lasts ___, then ___.
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3--4 years
progresses to accelerated or blast phase |
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allogeneic SC transplants work best if ___ (3)
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chronic phase
soon after dx < 40 y.o. |
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3 txs for CML
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IFNa
autologous SCT imatinib mesylate |
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IFNa is sometimes given with ___
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cytarabine
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imatinib mechanism
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blocks ATP binding site on Abl
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imatinib is aka
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glivec
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tx for accelerated phase
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imatinib
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when in blast crisis, CML is treated like ___. prognosis is ___.
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acute leukemia
bad |
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2 drugs for palliative CML tx
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busulfan
hydroxyurea |