MYD88 L265P present in >90% of WM. Activates NF-κB via BTK/IRAK signaling — rationale for...
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | BMA-MYD88-L265P-WM |
|---|---|
| Type | Actionability |
| Status | reviewed 2026-04-27 | pending_clinical_signoff | actionability review required |
| Diseases | DIS-WM |
| Sources | SRC-CIVIC SRC-ESMO-WM-2024 SRC-NCCN-BCELL-2025 |
Actionability Facts
| Biomarker | BIO-MYD88-L265P |
|---|---|
| Variant | L265P |
| Disease | DIS-WM |
| ESCAT tier | IA |
| Recommended combinations | zanubrutinib (preferred per ASPEN — fewer cardiac AE vs ibrutinib), ibrutinib monotherapy, ibrutinib + rituximab, BR (chemo-immuno alternative) |
| Evidence summary | MYD88 L265P present in >90% of WM. Activates NF-κB via BTK/IRAK signaling — rationale for BTKi. Ibrutinib monotherapy (iNNOVATE substudy 2 / arm A — Treon et al. NEJM 2015 R/R; iNNOVATE — Dimopoulos et al. NEJM 2018 1L+R/R with rituximab) and zanubrutinib (ASPEN — Tam et al. Blood 2020) FDA-approved. CXCR4 WHIM-like co-mutation reduces but does not abolish response. |
Notes
ESCAT IA. OncoKB Level 1. CXCR4 WHIM-like mutation testing recommended as it modulates BTKi response. MYD88-WT WM (~10%) responds less well to BTKi.
Used By
No reverse references found in the YAML corpus.