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BRAF V600E is the defining molecular lesion of classic hairy cell leukemia (~100% of cHCL...

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

IDBMA-BRAF-V600E-HCL
TypeActionability
Statusreviewed 2026-04-27 | pending_clinical_signoff | actionability review required
DiseasesDIS-HCL
SourcesSRC-CIVIC SRC-NCCN-BCELL-2025

Actionability Facts

BiomarkerBIO-BRAF-V600E
VariantV600E
DiseaseDIS-HCL
ESCAT tierIB
Recommended combinationsvemurafenib monotherapy (R/R cHCL), vemurafenib + rituximab (consolidation / R/R), dabrafenib + trametinib (alternative)
Evidence summaryBRAF V600E is the defining molecular lesion of classic hairy cell leukemia (~100% of cHCL; absent in HCL-variant). Vemurafenib monotherapy yields CR ~35% / ORR ~96% in relapsed/refractory cHCL (Tiacci et al. NEJM 2015). Vemurafenib + rituximab gives durable CR in ~87% (Tiacci et al. NEJM 2021). Dabrafenib + trametinib also active. Used as salvage after purine-analog failure or in cladribine-ineligible patients.

Notes

ESCAT IB. OncoKB Level 2. Frontline cHCL still cladribine ± rituximab (MDACC) — BRAFi reserved for relapsed/refractory or unfit patients. HCL-variant lacks BRAF V600E and is BRAFi-resistant.

Used By

No reverse references found in the YAML corpus.