Essential thrombocythemia: Difference between revisions
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* Must distinguish from other myelodysplastic disorders (especially polycythemia vera) as well as from secondary causes of thrombocytosis |
* Must distinguish from other myelodysplastic disorders (especially polycythemia vera) as well as from secondary causes of thrombocytosis |
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| − | == Clinical |
+ | == Clinical Manifestations == |
* History |
* History |
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Revision as of 13:07, 19 July 2020
Definition
- Myelodysplastic disorder of the thrombocytopoeitic stem cells causing a clonal proliferation of thrombocytes
Differential Diagnosis
- Must distinguish from other myelodysplastic disorders (especially polycythemia vera) as well as from secondary causes of thrombocytosis
Clinical Manifestations
- History
- History of thrombosis or bleeding
- Signs & Symptoms
- Digital ischemia
- Cerebrovascular ischemia
- High risk for vascular events if:
- History of thrombosis or bleeding
- Increased cardiovascular risk
- Older than 60 years
Management
- Acute
- If digital or cerebrovascular ischemia, rapid cytoreduction with plateletpheresis
- Chronic
- If high risk, requires cytoreduction and aspirin:
- First-line: hydroxyurea
- Alternative: anagrelide
- Pregnancy: interferon alfa
- If >1.5m platelets, requires cytoreduction alone
- If high risk, requires cytoreduction and aspirin:
