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NOTCH1 activating mutations (~10-15% of CLL) — adverse prognostic; reduced response to an...

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

IDBMA-NOTCH1-ACTIVATING-CLL
TypeActionability
Statusreviewed 2026-04-27 | pending_clinical_signoff | actionability review required
DiseasesDIS-CLL
SourcesSRC-CIVIC SRC-ESMO-CLL-2024 SRC-NCCN-BCELL-2025

Actionability Facts

BiomarkerBIO-NOTCH1-MUTATION
Variantactivating (PEST domain truncating, c.7541_7542delCT canonical)
DiseaseDIS-CLL
ESCAT tierIIIA
Recommended combinationsvenetoclax + obinutuzumab (CLL14), acalabrutinib monotherapy, zanubrutinib monotherapy
Contraindicated monotherapychlorambucil + obinutuzumab (relatively reduced anti-CD20 benefit per NOTCH1-mut subgroup)
Evidence summaryNOTCH1 activating mutations (~10-15% of CLL) — adverse prognostic; reduced response to anti-CD20 mAb (rituximab, obinutuzumab) in some studies. Venetoclax-based fixed-duration (CLL14) and BTKi continuous remain effective. Not directly targeted; selects venetoclax-based therapy when present.

Notes

ESCAT IIIA. NOTCH1 mutation identifies a subgroup that may be especially well-served by venetoclax-based regimens vs anti-CD20-based chemoimmuno.

Used By

No reverse references found in the YAML corpus.