Onco Wiki / Maladie

Myélofibrose primaire (DIPSS-Plus stratifié)

Vue déterministe de l'entité source de la LJAM. L'autorité clinique demeure avec les ID de la source cités et l'état d'approbation de l ́examinateur.

IDDIS-PMF
TypeMaladie
Alias
Myélofibrose idiopathiqueMyélofibrose avec métaplasie myéloïdePMFMyélofibrose primaireMyélofibrose primaireПервинний мієлофіброзХвороба Вакеза-Османд
Étatreviewed 2026-04-25 | pending_clinical_signoff
MaladiesAucune déclaration
SourcesSRC-COMFORT-I-VERSTOVSEK-2012 SRC-DIPSS-PLUS-GANGAT-2011 SRC-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_morphology9961/3
icd_o_3_topography["C42.1"]
icd_10D47.4
who_classificationPrimary myelofibrosis (PMF) — Ph-negative myeloproliferative neoplasm; prefibrotic/early stage and overt fibrotic stage (per WHO 5th ed.)

Capacités

Notes

Risk-stratified archetype using DIPSS-Plus. Clinical decision flow: (1) Transplant-eligibility assessment for intermediate-2 / high-risk; (2) symptomatic splenomegaly / constitutional symptoms → ruxolitinib (COMFORT-I/II) — momelotinib if anemia is dominant problem (MOMENTUM); fedratinib 2L (FREEDOM-2); pacritinib for severe thrombocytopenia (PERSIST). Anemia management as dedicated track (transfusion, ESA in selected, danazol). NEVER abrupt-discontinue ruxolitinib (cytokine rebound). STUB — requires clinical co-lead signoff before publication. Myeloid expansion (heme).

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Pertinence thérapeutique

Algorithmes

Maladie

Indication

Questionnaires

Signal d’alerte

Sources