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18 Cards in this Set
- Front
- Back
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main features of polycythemia vera (PV)?
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elevated RBC mass due to autonomic RBC production
accompanied by increased granulocytic and platelet production JAK2 mutation in 90% thrombosis/bleeding |
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associated disorders in PV?
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hyperhomocysteinemia --> RF for thrombosis
acquired vWF syndrome due to absorption of the factor onto the platelets increased risk for peptic ulcer disease 3- to 5-fold <-- histamine levels increased |
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what is the incidence of PV?
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2-3/100'000, thus most common of the myeloproliferative neoplasms
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symptoms of PV?
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various neurologic symptoms due to impairment of microcirculation
plethora pruritus, aquagenic |
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frequency of hepatosplenomegaly in PV?
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splenomegaly 75%
hepatomegaly 30% |
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principal diagnostic criteria for PV?
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major and minor
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major criteria in PV?
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Hb >185g/l in men, >165 g/l in women
presence of JAK2V617F mutation |
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minor criteria in PV?
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1 BM trilineage myeloproliferation
2 subnormal serum Epo level 3 EEC growth (endogenous erythroid colony growth) |
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criteria for diagnosis of PV?
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both major plus one minor
first major plus two minor |
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first line therapy principles in PV?
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phlebotomy
Interferon-α ASS 100 mg bei Thrombozytose, Reservemittel bei Thrombozytose ist Anagrelid (Xagrid) |
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second line therapy in PV?
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hydroxyurea
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indications for second line therapy in PV?
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s/p thrombembolic complications
uncontrolled myeloproliferation symptomatic splenomegaly |
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phases in PV?
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hyperproliferative early phase
pancytopenic late phase |
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median survival in PV?
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2 years without treatment
10-20 years with treatment |
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transformation of PV which disorders per 20 years?
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acute leucemia in 15%/ 20 years
osteomyelofibrosis 10%/ 20 years |
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4 major fatality causes in PV?
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thrombembolic
hemorrhagic diathesis development of MDS or acute leukemia development of osteomyelofibrosis |
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PV is the most common of what disorder?
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hepatic vein thrombosis
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what is the median age at diagnosis in PV?
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60 years
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