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Essential thrombocytosis
From WikEM
Contents
Background
- Rare, chronic myeloproliferative neoplasm
- Abnormal proliferation is seen in megakaryocytic line only
- Typically in elderly
- Generally asymptomatic
Clinical Features
- Leukocytosis
- Anemia
- Splenomegaly
- Clotting or bleeding
Differential Diagnosis
Evaluation
Workup
- CBC
- Chem 7
- ESR
- Bone marrow biopsy sometimes needed
Evaluation[1]
Per the WHO guidelines, diagnosis of ET requires all of the following:
Major criteria |
---|
Thrombocytosis ≥ 450 x 109L |
Megakaryocyte proliferation with little to no granulocyte or erythroid proliferation |
Not meeting criteria for CML, PV, PMF, MDS or other myeloid neoplasm |
No evidence of reactive thrombocytosis or presence of JAK2V617F or other clonal marker |
Management
- ASA 81mg
- Myelosuppressive agents (hydroxyurea, interferon alpha)
- Anagrelide
Disposition
Also See
References
- ↑ Tefferi, A, et al. The 2008 World Health Organization classification system for myeloproliferative neoplasms: order out of chaos. Cancer. 2009; 115(17):3842-7,