CALR exon 9 indels sont le deuxième pilote le plus commun dans PMF (~25%) et un WHO 2022 / ICC 2...
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.
| ID | BMA-CALR-PMF |
|---|---|
| Type | Pertinence thérapeutique |
| État | reviewed 2026-04-27 | pending_clinical_signoff | actionability review required |
| Maladies | DIS-PMF |
| Sources | SRC-CIVIC SRC-COMFORT-I-VERSTOVSEK-2012 SRC-DIPSS-PLUS-GANGAT-2011 SRC-ESMO-MPN-2015 SRC-JAKARTA2-HARRISON-2017 SRC-MOMENTUM-VERSTOVSEK-2023 SRC-NCCN-MPN-2025 |
Faits relatifs à l ' action en justice
| Biomarqueur | BIO-CALR |
|---|---|
| Variante | exon 9 indels — type-1/type-1-like (dépression de 52 pb) et type-2/type-2-lime (5 pb d'insertion); ~25% de la myélofibrose primaire |
| Maladie | DIS-PMF |
| Niveau ESCAT | IA |
| Combinaisons recommandées | ruxolitinib monotherapy (intermediate-2 / high-risk per SRC-COMFORT-I-VERSTOVSEK-2012), fedratinib monotherapy (post-ruxolitinib failure per SRC-JAKARTA2-HARRISON-2017), momelotinib monotherapy (MF + anemia per SRC-MOMENTUM-VERSTOVSEK-2023), allogeneic HCT (transplant-eligible higher-risk per SRC-NCCN-MPN-2025, SRC-DIPSS-PLUS-GANGAT-2011) |
| Résumé des faits | CALR exon 9 indels are the second most common driver in PMF (~25%) and a WHO 2022 / ICC 2022 major diagnostic criterion in JAK2/MPL- negative cases (per SRC-NCCN-MPN-2025, SRC-ESMO-MPN-2015). CALR type-1 mutation in PMF is associated with better OS and lower leukemic transformation risk than JAK2 V617F or triple-negative PMF, integrated into MIPSS70+v2 risk model. Treatment is genotype- agnostic JAK-inhibitor therapy by symptom / risk score: ruxolitinib for symptomatic intermediate-2/high-risk (COMFORT-I extends to all driver genotypes); fedratinib for ruxolitinib failure (JAKARTA2); momelotinib for anemic patients (MOMENTUM); allogeneic HCT for transplant-eligible higher-risk patients per SRC-NCCN-MPN-2025. |
Notes
ESCAT IA / OncoKB Level 1 — diagnostic criterion + risk stratifier (favorable in MIPSS70+v2 when type-1). JAK inhibitors are mutation- agnostic (downstream JAK-STAT inhibition). Source-gap: SRC-NCCN-MPN-2025 STUB.
Utilisé par
No reverse references found in the YAML corpus.