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23 Cards in this Set
- Front
- Back
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what are the primary features of essential thrombocytosis (ET)?
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elevated platelet count in abscence of other causes
normal red blood cell mass |
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pathophysiology of ET?
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bone marrow megakaryocytes form colonies in abscence of exogenous thrombopoietin
JAK-2 mutation in 50% |
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what is the median age at diagnosis in ET?
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60 years
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proportion of asymptomatic patient in ET?
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one third
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what are the symptoms and signs of ET?
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thrombosis, sometimes in unusual sites
erythromelalgia = painful burning of hans with erythema --> prompt relief with aspirin bleeding is typically mucosal |
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primary site of bleeding in ET?
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gastrointestinal tract
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platelet count in ET when bleeding occurs?
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generally > 1 million/ul
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frequency of hepatosplenomegaly in ET?
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50% splenomegaly
20% hepatomegaly |
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differential diagnosis to ET?
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1. reactive thrombocytosis, most < 1'000'000/ul
2. other MPN |
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principles of therapy in ET?
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according to risc stratification
high, intermediate and low-risk patients |
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definition of high risk patients in ET?
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age >60
thrombembolic complications or severe bleeding platelets >1.5 mio/ul |
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therapy principles in high risk patients in ET?
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Hydroxyurea (Litalir)
Anagrelid (Xagrid) --> Reifungshemmung Megakaryozyten in KM Interferon-α (Roferon-A, Intron A) |
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definition of intermediate risk patients in ET?
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presence of cardiovascular risk factor(s)
--> ASS 100 mg |
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definition of low-risk patients in ET?
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age<60
asymptomatic platelets < 1.5 mio/ul |
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cause of death in ET?
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major cause of morbidity and mortality is arterial thrombosis
transformation to AML in up to 5%, risk higher in those with chemotherapy |
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severity of bleeding in ET?
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usually not severe, rarely transfusions necessary
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what are the characteristics of the white blood count in ET?
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often mildly elevated, usually not above 30'000, with some immature myeloid forms
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what are the characteristics of the platelet count in ET?
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markedly elevated, may be over 2'000'000
large platelets, but no giant degranulated forms like in myelofibrosis |
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what are the characteristics of the red blood count in ET?
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normal!
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objects of therapy in ET?
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control of platelet count first with hydroxyurea, otherwise with anagrelide
control of vasomotor symptoms with aspirin 100 mg |
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what is the course in ET?
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15% risk of transformation to AMM after 15 years
1-5% risk of transformation to acute leukemia over 20 years |
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what is the M:F ratio in ET?
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M>F 1.5:1, but in young people more often in young women
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what is the incidence of ET?
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1/100'000
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