CALR exon 9 indels are the second most common driver in ET (~25-30%) and a WHO 2022 / ICC...
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | BMA-CALR-ET |
|---|---|
| Type | Actionability |
| Status | reviewed 2026-04-27 | pending_clinical_signoff | actionability review required |
| Diseases | DIS-ET |
| Sources | SRC-CIVIC SRC-ESMO-MPN-2015 SRC-NCCN-MPN-2025 SRC-PT1-HARRISON-2005 |
Actionability Facts
| Biomarker | BIO-CALR |
|---|---|
| Variant | exon 9 indels — type-1/type-1-like (52-bp deletion) and type-2/type-2-like (5-bp insertion); ~25-30% of essential thrombocythemia |
| Disease | DIS-ET |
| ESCAT tier | IA |
| Recommended combinations | 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) |
| Evidence summary | 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.
Used By
No reverse references found in the YAML corpus.