ALK-positive ALCL (typically NPM1-ALK t(2;5)) is generally cured by CHOP-based regimens w...
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | BMA-ALK-FUSION-ALCL |
|---|---|
| Type | Actionability |
| Status | reviewed 2026-04-27 | pending_clinical_signoff | actionability review required |
| Diseases | DIS-ALCL |
| Sources | SRC-CIVIC SRC-ESMO-PTCL-2024 SRC-NCCN-BCELL-2025 |
Actionability Facts
| Biomarker | BIO-ALK-FUSION |
|---|---|
| Variant | NPM1-ALK and other ALK fusions |
| Disease | DIS-ALCL |
| ESCAT tier | IIA |
| Recommended combinations | brentuximab vedotin + cyclophosphamide/doxorubicin/prednisone (BV-CHP, 1L), crizotinib monotherapy (R/R, especially pediatric), alectinib / lorlatinib (off-label salvage) |
| Evidence summary | ALK-positive ALCL (typically NPM1-ALK t(2;5)) is generally cured by CHOP-based regimens with brentuximab vedotin (ECHELON-2: BV-CHP). For relapsed/refractory ALK+ ALCL, crizotinib monotherapy achieves high response rates (Gambacorti-Passerini 2014; pediatric COG NCT00939770). ALK-TKIs are an established salvage option but not 1L. |
Notes
ESCAT IIA — strong retrospective + single-arm evidence for ALK-TKI in R/R ALK+ ALCL. ALK status by IHC (ALK1 antibody) is mandatory for ALCL diagnosis per WHO.
Used By
Actionability
BMA-CD30-ALCL- CD30 expression is a defining feature of anaplastic large-cell lymphoma (sALCL); brentuxi...