Primary Myelofibrosis (DIPSS-Plus stratified)
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-PMF |
|---|---|
| Type | Disease |
| Aliases | Idiopathic myelofibrosisMyelofibrosis with myeloid metaplasiaPMFPrimary MyelofibrosisPrimary myelofibrosisПервинний мієлофіброзХвороба Вакеза-Османд |
| Status | reviewed 2026-04-25 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | SRC-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.
| icd_o_3_morphology | 9961/3 |
|---|---|
| icd_o_3_topography | ["C42.1"] |
| icd_10 | D47.4 |
| who_classification | Primary myelofibrosis (PMF) — Ph-negative myeloproliferative neoplasm; prefibrotic/early stage and overt fibrotic stage (per WHO 5th ed.) |
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).
Used By
Actionability
BMA-CALR-PMF- CALR exon 9 indels are the second most common driver in PMF (~25%) and a WHO 2022 / ICC 2...BMA-JAK2-V617F-PMF- JAK2 V617F is one of three defining drivers in primary myelofibrosis (~50-60%; CALR ~25%,...
Algorithms
ALGO-PMF-1L- ALGO-PMF-1LALGO-PMF-2L- ALGO-PMF-2L
Disease
DIS-AML- Acute Myeloid Leukemia (non-APL)DIS-ET- Essential ThrombocythemiaDIS-PV- Polycythemia Vera
Indication
IND-MPN-SECONDARY-LEUKEMIA-PREVENTION-OBSERVATION- IND-MPN-SECONDARY-LEUKEMIA-PREVENTION-OBSERVATIONIND-MPN-SECONDARY-LEUKEMIA-PREVENTION-SURVEILLANCE- IND-MPN-SECONDARY-LEUKEMIA-PREVENTION-SURVEILLANCEIND-PMF-1L-OBSERVATION- IND-PMF-1L-OBSERVATIONIND-PMF-1L-RUXOLITINIB- IND-PMF-1L-RUXOLITINIBIND-PMF-2L-FEDRATINIB- IND-PMF-2L-FEDRATINIBIND-PMF-ALLOHCT-HIGH-RISK- IND-PMF-ALLOHCT-HIGH-RISKIND-PMF-MOMELOTINIB-ANEMIA- IND-PMF-MOMELOTINIB-ANEMIA
Questionnaires
QUEST-PMF-1L-STUB- Primary Myelofibrosis — first line
Red flag
RF-DIPSS-PLUS-HIGH- Primary myelofibrosis with DIPSS-Plus intermediate-2 or high risk — alloHCT referral mand...RF-MPN-SECONDARY-LEUKEMIA-PREVENTION- Myeloproliferative neoplasm (polycythemia vera [PV], essential thrombocythemia [ET], or p...RF-PMF-ANEMIA-DOMINANT- PMF with anemia as dominant clinical problem (Hb <10 g/dL OR transfusion-dependent) — mom...RF-PMF-BLAST-PROGRESSION- PMF progressing toward AML (blasts in PB ≥10% accelerated, ≥20% blast-phase / post-MPN AM...RF-PMF-FRAILTY-AGE- PMF patient elderly or frail (age ≥75, ECOG ≥3, or HCT-CI ≥4) — alloHCT off the table; sy...RF-PMF-HIGH-RISK-DIPSS- PMF intermediate-2 or high risk by DIPSS-Plus — alloHCT referral mandatory in eligible pa...RF-PMF-INFECTION-SCREENING- PMF patient with positive HBV / HCV / HIV serology, latent TB, or active uncontrolled inf...RF-PMF-ORGAN-DYSFUNCTION- PMF patient with organ dysfunction limiting JAK-inhibitor or alloHCT eligibility: severe...
Sources
SRC-COMFORT-I-VERSTOVSEK-2012- A double-blind, placebo-controlled trial of ruxolitinib for myelofibrosisSRC-DIPSS-PLUS-GANGAT-2011- DIPSS plus: a refined Dynamic International Prognostic Scoring System for primary myelofi...SRC-ESMO-MPN-2015- Philadelphia-negative chronic myeloproliferative neoplasms: ESMO Clinical Practice Guidel...SRC-JAKARTA2-HARRISON-2017- Janus kinase inhibition with fedratinib in patients with myelofibrosis previously treated...SRC-MOMENTUM-VERSTOVSEK-2023- Momelotinib versus danazol in symptomatic patients with anaemia and myelofibrosis (MOMENT...