Waldenström Macroglobulinemia / Lymphoplasmacytic Lymphoma
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-WM |
|---|---|
| Type | Disease |
| Aliases | LPLWMМакроглобулінемія Вальденстрема / Лімфоплазмоцитарна лімфома |
| 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 | 9671/3 |
|---|---|
| icd_10 | C88.0 |
| who_classification | Mature B-cell neoplasms — Lymphoplasmacytic lymphoma per WHO 5th ed. |
Notes
Only NHL with defining "must-have" mutation (MYD88 L265P) — strongly predicts BTKi response. CXCR4 status affects BTKi depth of response (WT > WHIM-mutated). Hyperviscosity emergency requires plasmapheresis before any cytotoxic therapy.
Used By
Actionability
BMA-CXCR4-WHIM-WM- CXCR4 WHIM-like mutations co-occur in ~30-40% of MYD88-L265P Waldenström macroglobulinemi...BMA-MYD88-L265P-WM- MYD88 L265P present in >90% of WM. Activates NF-κB via BTK/IRAK signaling — rationale for...
Algorithms
ALGO-WM-1L- ALGO-WM-1LALGO-WM-2L- ALGO-WM-2L
Indication
IND-WM-1L-BTKI- IND-WM-1L-BTKIIND-WM-1L-DRC- IND-WM-1L-DRCIND-WM-2L-CARFILZOMIB-CXCR4MUT- IND-WM-2L-CARFILZOMIB-CXCR4MUTIND-WM-2L-VRD- IND-WM-2L-VRDIND-WM-2L-ZANUBRUTINIB- IND-WM-2L-ZANUBRUTINIB
Questionnaires
QUEST-WM-1L-STUB- Waldenström Macroglobulinemia / Lymphoplasmacytic Lymphoma — first line
Red flag
RF-WM-CXCR4-WHIM-MUTANT- CXCR4 WHIM-like nonsense or frameshift mutation in the C-terminal regulatory domain (e.g....RF-WM-FRAILTY-AGE- Frailty profile influencing WM regimen selection: ECOG ≥3, OR (age ≥75 with G8 ≤14), OR c...RF-WM-HIGH-RISK-BIOLOGY- Waldenström with adverse molecular biology: MYD88-wildtype (poor BTKi response — ibrutini...RF-WM-HYPERVISCOSITY- Waldenström with symptomatic hyperviscosity syndrome (serum viscosity >4 cp typical, or s...RF-WM-INFECTION-SCREENING- Active or latent infection requiring resolution / prophylaxis before initiating WM therap...RF-WM-MYD88-L265P-ACTIONABLE- MYD88 L265P is the hallmark mutation of Waldenström macroglobulinemia, present in >90% of...RF-WM-TRANSFORMATION-PROGRESSION- Waldenström with clinical / biochemical features concerning for transformation to DLBCL:...
Sources
SRC-BRUIN-MATO-2021- Pirtobrutinib in relapsed or refractory B-cell malignancies (BRUIN): a phase 1/2 studySRC-ESMO-WM-2024- ESMO Clinical Practice Guideline on Waldenström MacroglobulinaemiaSRC-INNOVATE-DIMOPOULOS-2018- Phase 3 Trial of Ibrutinib plus Rituximab in Waldenström's Macroglobulinemia (iNNOVATE)