CALR exon 9 indels are the second most common driver in ET (~25-30%) and a WHO 2022 / ICC...

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ID	`BMA-CALR-ET`
Type	Pertinence thérapeutique
État	reviewed 2026-04-27 \| pending_clinical_signoff \| actionability review required
Maladies	`DIS-ET`
Sources	`SRC-CIVIC` `SRC-ESMO-MPN-2015` `SRC-NCCN-MPN-2025` `SRC-PT1-HARRISON-2005`

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 insertion de pb); ~25-30% de thrombocythémie essentielle
Maladie	`DIS-ET`
Niveau ESCAT	IA
Combinaisons recommandées	observation (very-low-risk CALR-mutated per SRC-ESMO-MPN-2015), low-dose aspirin (low-risk per SRC-NCCN-MPN-2025), hydroxyurea + aspirin (high-risk 1L per SRC-PT1-HARRISON-2005), interferon-alpha (preferred for younger high-risk per SRC-NCCN-MPN-2025)
Résumé des faits	CALR exon 9 indels are the second most common driver in ET (~25-30%) and a WHO 2022 / ICC 2022 major diagnostic criterion in JAK2/MPL- negative cases (per SRC-NCCN-MPN-2025, SRC-ESMO-MPN-2015). CALR- mutated ET has lower thrombosis risk than JAK2-mutated ET, which feeds into IPSET-thrombosis stratification: very-low-risk CALR patients may be managed with observation alone; otherwise risk- stratified management identical to JAK2-positive ET (low-risk → aspirin; high-risk → hydroxyurea/IFN per SRC-PT1-HARRISON-2005).

Notes

ESCAT IA / OncoKB Level 1 — diagnostic criterion. CALR type-1 (52-bp deletion) is more common in PMF and confers better OS than type-2 per several retrospective series; type-2 is enriched in ET. CALR- specific therapeutics (anti-CALR monoclonal antibodies, e.g. INCA 033989) are in early-phase trials only; no in-repo source ingested yet. Source-gap: SRC-NCCN-MPN-2025 STUB.

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