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essential-thrombocythemia
Essential Thrombocythemia
Essential thrombocythemia is a JAK2/CALR/MPL-driven MPN with megakaryocytic hyperplasia and thrombocytosis; JAK2 V617F ~55-60%; CALR ~20-25%; MPL ~5-8%; risk-stratified aspirin ± hydroxyurea; anagrelide second-line; post-ET MF (~1-2%) and AML (<1%) transformation risk.
Entry Metadata
| Field | Value |
|---|---|
| ID | essential-thrombocythemia |
| Name | Essential Thrombocythemia |
| Status | draft |
| Last reviewed | 2026-06-06 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
connects-toMpl
connects-toJak2
connects-toCalr
connects-toTgf Beta
connects-toVon Willebrand Factor
connects-toPolycythemia Vera
connects-toPlatelet
connects-toDisseminated Intravascular Coagulation
connects-toBone Marrow
connects-toSpleen
connects-toMyeloproliferative Neoplasms
connects-toMyelofibrosis
connects-toVenous Thromboembolism
Sources
- Harrison CN, Campbell PJ, Buck G, et al. Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia. N Engl J Med. 2005;353(1):33-45. · PubMed 16000354
- Barbui T, Finazzi G, Carobbio A, et al. Development and validation of an International Prognostic Score of thrombosis in World Health Organization-essential thrombocythemia (IPSET-thrombosis). Blood. 2012;120(26):5128-5133. · PubMed 23086758