Hairy Cell Leukemia
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-HCL |
|---|---|
| Type | Disease |
| Aliases | HCLВолосатоклітинний лейкоз |
| Status | reviewed 2026-04-25 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | SRC-NCCN-BCELL-2025 |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 9940/3 |
|---|---|
| icd_10 | C91.4 |
| who_classification | Mature B-cell leukemias — Hairy cell leukemia per WHO 5th ed. |
Notes
Watchful waiting for asymptomatic; treatment indication: significant cytopenia (Hb <11, plt <100K, ANC <1K), symptomatic splenomegaly, recurrent infections. BRAF V600E targeted therapy (vemurafenib) emerging for relapsed/refractory.
Used By
Actionability
BMA-BRAF-V600E-HCL- BRAF V600E is the defining molecular lesion of classic hairy cell leukemia (~100% of cHCL...BMA-MAP2K1-HCL- Hairy cell leukemia variant (HCL-V) is a rare splenic B-cell lymphoma (~10% of HCL cases)...
Algorithms
ALGO-HCL-1L- ALGO-HCL-1LALGO-HCL-2L- ALGO-HCL-2L
Indication
IND-HCL-1L-CLADRIBINE- IND-HCL-1L-CLADRIBINEIND-HCL-1L-PENTOSTATIN- IND-HCL-1L-PENTOSTATININD-HCL-2L-VEMURAFENIB- IND-HCL-2L-VEMURAFENIB
Questionnaires
QUEST-HCL-1L-STUB- Hairy Cell Leukemia — first line
Red flag
RF-HCL-FRAILTY-AGE- Age >75 + ECOG ≥2 OR significant comorbidity — pentostatin's weekly schedule allows finer...RF-HCL-HIGH-RISK-BIOLOGY- BRAF V600E wild-type variant HCL (HCL-v) OR IGHV4-34 expression — purine analogs less eff...RF-HCL-INFECTION-SCREENING- Active uncontrolled infection at HCL diagnosis — defer purine analog (cladribine/pentosta...RF-HCL-ORGAN-DYSFUNCTION- Renal dysfunction (CrCl <60 mL/min) — cladribine clearance reduced; dose adjustment or pe...RF-HCL-TRANSFORMATION-PROGRESSION- Failure to achieve CR after 2 courses cladribine OR early relapse (<2 years) — switch to...