Onco Wiki / Disease

Essential Thrombocythemia

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

IDDIS-ET
TypeDisease
Aliases
ETEssential thrombocythaemiaPrimary thrombocythemiaЕсенціальна тромбоцитемія
Statusreviewed 2026-04-25 | pending_clinical_signoff
DiseasesNone declared
SourcesSRC-ESMO-MPN-2015 SRC-NCCN-MPN-2025

Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.

Source YAML

icd_o_3_morphology9962/3
icd_o_3_topography["C42.1"]
icd_10D47.3
who_classificationEssential thrombocythaemia (ET) — Ph-negative myeloproliferative neoplasm (per WHO 5th ed.)

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Notes

Authored as separate disease from PV (see DIS-PV.notes for rationale). Treatment: low-risk ET → ASA only (avoid in extreme thrombocytosis with acquired vWD); high-risk → HU 1L; anagrelide 2L (PT-1 trial — non-inferior on thrombosis but more arterial events + bleeding + MF progression). PreT-PMF ("prefibrotic primary myelofibrosis") is increasingly distinguished from ET on trephine — has worse prognosis and behaves more like PMF; current scope: standard ET only. STUB — requires clinical co-lead signoff before publication. Myeloid expansion (heme).

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