Mantle Cell Lymphoma
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-MCL |
|---|---|
| Type | Disease |
| Aliases | MCLЛімфома з клітин зони мантії |
| Status | reviewed 2026-04-25 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | SRC-EHA-WORKUP-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 | 9673/3 |
|---|---|
| icd_10 | C83.1 |
| who_classification | Mature B-cell neoplasms — Mantle cell lymphoma per WHO 5th ed. |
Notes
CD5+ CD20+ CD23- cyclin D1+ SOX11+ classic phenotype. Strong overlap with CLL on flow (both CD5+ B-cell) — IHC + FISH t(11;14) distinguish. Treatment paradigm: fitness-based — fit/transplant-eligible get intensive chemoimmuno + autoSCT; unfit get BTKi-based (acalabrutinib, zanubrutinib) +- rituximab. TP53-mutant: poor response to chemoimmuno; go directly BTKi-based.
Used By
Actionability
BMA-ATM-LOSS-MCL- ATM is the most common somatic mutation in MCL (~40-50%). Not directly therapy-selecting;...BMA-BCL2-EXPRESSION-MCL- High BCL2 in MCL universal (cyclin D1-driven survival pathway). Venetoclax + ibrutinib (A...BMA-BCL2-REARRANGEMENT-MCL- BCL2 rearrangement in MCL is very rare and usually denotes composite lymphoma or transfor...BMA-CCND1-IHC-MCL- Cyclin D1 IHC is a defining diagnostic marker of MCL (positive in >95%; SOX11 used in cyc...BMA-CCND1-T1114-MCL- t(11;14) IGH/CCND1 is the defining genetic lesion of MCL — drives cyclin D1 overexpressio...BMA-NOTCH1-ACTIVATING-MCL- NOTCH1 mutations in ~10-15% of MCL — adverse prognostic; not directly targeted. BTKi-base...BMA-TP53-MUT-MCL- TP53-mut MCL — predicts chemoimmuno failure. Acalabrutinib + rituximab (TRIANGLE / ECHO)...BMA-TP53-R175H-MCL- TP53 hotspot in MCL — chemoimmuno failure predictor; acalabrutinib-based 1L preferred. Ho...BMA-TP53-R248Q-MCL- TP53 hotspot in MCL — chemoimmuno failure predictor; acalabrutinib-based 1L preferred. Ho...BMA-TP53-R273H-MCL- TP53 hotspot in MCL — chemoimmuno failure predictor; acalabrutinib-based 1L preferred. Ho...BMA-TP53-R282W-MCL- TP53 hotspot in MCL — chemoimmuno failure predictor; acalabrutinib-based 1L preferred. Ho...
Algorithms
ALGO-MCL-1L- ALGO-MCL-1LALGO-MCL-2L- ALGO-MCL-2LALGO-MCL-3L- ALGO-MCL-3L
Disease
DIS-FL- Follicular Lymphoma
Indication
IND-MCL-1L-BTKI-R- IND-MCL-1L-BTKI-RIND-MCL-1L-INTENSIVE- IND-MCL-1L-INTENSIVEIND-MCL-2L-ACALABRUTINIB- IND-MCL-2L-ACALABRUTINIBIND-MCL-3L-BREXUCEL-CART- IND-MCL-3L-BREXUCEL-CARTIND-MCL-3L-LISOCEL- IND-MCL-3L-LISOCELIND-MCL-3L-PIRTOBRUTINIB- IND-MCL-3L-PIRTOBRUTINIBIND-MCL-POST-INDUCTION-RITUXIMAB-MAINTENANCE- IND-MCL-POST-INDUCTION-RITUXIMAB-MAINTENANCE
Questionnaires
QUEST-MCL-1L-STUB- Mantle Cell Lymphoma — first line
Red flag
RF-MCL-BLASTOID-OR-TP53- Mantle cell lymphoma with blastoid / pleomorphic morphology OR TP53 mutation OR del(17p)...RF-MCL-BLASTOID-VARIANT- Blastoid or pleomorphic histologic variant of mantle cell lymphoma — ~10-15% of MCL at di...RF-MCL-FRAILTY-AGE- Frailty profile precluding intensive MCL induction (Nordic R-MaxiCHOP/AraC, R-DHAP) or au...RF-MCL-INFECTION-SCREENING- Active or latent infection requiring resolution / prophylaxis before initiating any anti-...RF-MCL-ORGAN-DYSFUNCTION- Baseline organ dysfunction precluding standard MCL induction (Nordic R-MaxiCHOP/AraC, R-D...RF-MCL-POST-BTKI-C481-ACTIONABLE- MCL with acquired BTK C481S (rarely C481R/F/Y) mutation after progression on covalent BTK...RF-MCL-TRANSFORMATION-PROGRESSION- MCL with morphologic progression to blastoid or pleomorphic variant (often with TP53 acqu...RF-MIPI-HIGH- Mantle cell lymphoma with MIPI high risk (MIPI ≥6.2 / simplified ≥6 points) — aggressive...RF-MIPI-LOW- Mantle cell lymphoma with MIPI low risk (MIPI <5.7 / 0-3 simplified points) — supports st...
Sources
SRC-BRUIN-MATO-2021- Pirtobrutinib in relapsed or refractory B-cell malignancies (BRUIN): a phase 1/2 studySRC-ESMO-LYMPHOMA-2025- Lymphomas: ESMO Clinical Practice Guideline for diagnosis, treatment and follow-upSRC-ESMO-MCL-2024- ESMO Clinical Practice Guideline on Mantle Cell LymphomaSRC-MOZ-UA-LYMPH-2013- Уніфікований клінічний протокол первинної, вторинної (спеціалізованої) медичної допомоги:...SRC-ZUMA-2-WANG-2020- KTE-X19 CAR T-Cell Therapy in Relapsed or Refractory Mantle-Cell Lymphoma