Splenic Marginal Zone Lymphoma
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-SPLENIC-MZL |
|---|---|
| Type | Disease |
| Aliases | SMZLСелезінкова лімфома маргінальної зони |
| Status | reviewed 2026-04-25 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | SRC-BSH-MZL-2024 SRC-ESMO-MZL-2024 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 | 9689/3 |
|---|---|
| icd_10 | C83.0 |
| who_classification | Mature B-cell neoplasms — Splenic marginal zone lymphoma per WHO 5th ed. |
Notes
Clinical hallmark: massive splenomegaly + cytopenias from hypersplenism (often with little lymphadenopathy). HCV testing MANDATORY at diagnosis — DAA can produce durable lymphoma regression. For HCV-negative, rituximab monotherapy or splenectomy are 1L options.
Used By
Actionability
BMA-MYD88-L265P-SPLENIC-MZL- MYD88 L265P found in ~5-10% of MZL (more in lymphoplasmacytoid variants; rare in classic...
Algorithms
ALGO-SMZL-1L- ALGO-SMZL-1LALGO-SMZL-2L- ALGO-SMZL-2L
Disease
DIS-HCV-MZL- HCV-associated Marginal Zone LymphomaDIS-NODAL-MZL- Nodal Marginal Zone Lymphoma
Indication
IND-SMZL-1L-HCV-POSITIVE- IND-SMZL-1L-HCV-POSITIVEIND-SMZL-1L-RITUXIMAB- IND-SMZL-1L-RITUXIMABIND-SMZL-2L-BR- IND-SMZL-2L-BR
Questionnaires
QUEST-SPLENIC-MZL-1L-STUB- Splenic Marginal Zone Lymphoma — first line
Red flag
RF-SMZL-FRAILTY-AGE- Age >80 OR ECOG ≥3 with significant comorbidity AND asymptomatic SMZL — watch-and-wait ma...RF-SMZL-HIGH-RISK-BIOLOGY- TP53 mutation, NOTCH2 mutation, or 7q deletion — aggressive SMZL variant with shorter PFS...RF-SMZL-INFECTION-SCREENING- HCV RNA PCR mandatory at SMZL diagnosis — HCV-driven SMZL responds to direct-acting antiv...RF-SMZL-ORGAN-DYSFUNCTION- Severe portal hypertension (varices, ascites, splenic vein thrombosis) OR Child-Pugh B/C...RF-SMZL-TRANSFORMATION-PROGRESSION- Histologic transformation to DLBCL (rapid progression, new constitutional symptoms, LDH s...