Essential thrombocythemia: Difference between revisions
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== Definition == |
== Definition == |
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* Myelodysplastic disorder of the thrombocytopoeitic stem cells causing a clonal proliferation of thrombocytes |
* [[Myelodysplastic disorder]] of the thrombocytopoeitic stem cells causing a clonal proliferation of thrombocytes |
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== Clinical Manifestations == |
== Clinical Manifestations == |
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** Increased cardiovascular risk |
** Increased cardiovascular risk |
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** Older than 60 years |
** Older than 60 years |
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== Management == |
== Management == |
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* Acute |
* Acute |
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** If digital or cerebrovascular ischemia, rapid cytoreduction with |
** If digital or cerebrovascular ischemia, rapid cytoreduction with platelet pheresis |
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* Chronic |
* Chronic |
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** If high risk, requires cytoreduction and aspirin: |
** If high risk, requires cytoreduction and [[aspirin]]: |
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*** First-line: hydroxyurea |
*** First-line: [[hydroxyurea]] |
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*** Alternative: anagrelide |
*** Alternative: [[anagrelide]] |
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*** Pregnancy: interferon alfa |
*** Pregnancy: [[interferon alfa]] |
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** If >1.5m platelets, requires cytoreduction alone |
** If >1.5m platelets, requires cytoreduction alone |
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Revision as of 14:24, 20 February 2022
Definition
- Myelodysplastic disorder of the thrombocytopoeitic stem cells causing a clonal proliferation of thrombocytes
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
Differential Diagnosis
- Must distinguish from other myelodysplastic disorders (especially polycythemia vera) as well as from secondary causes of thrombocytosis
Management
- Acute
- If digital or cerebrovascular ischemia, rapid cytoreduction with platelet pheresis
- 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:
