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HGBL with MYC + BCL2 (and/or BCL6) rearrangements ('double-hit' / 'triple-hit') has aggre...

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

IDBMA-MYC-REARRANGEMENT-HGBL-DH
TypeActionability
Statusreviewed 2026-04-27 | pending_clinical_signoff | actionability review required
DiseasesDIS-HGBL-DH
SourcesSRC-CIVIC SRC-ESMO-DLBCL-2024 SRC-NCCN-BCELL-2025

Actionability Facts

BiomarkerBIO-DOUBLE-HIT
VariantMYC + BCL2 (DH) or MYC + BCL2 + BCL6 (TH)
DiseaseDIS-HGBL-DH
ESCAT tierIIA
Recommended combinationsDA-EPOCH-R (1L preferred), pola-R-CHP (1L alternative), axi-cel / liso-cel (2L+)
Contraindicated monotherapyR-CHOP alone (undercure in DH/TH per retrospective series)
Evidence summaryHGBL with MYC + BCL2 (and/or BCL6) rearrangements ('double-hit' / 'triple-hit') has aggressive course with R-CHOP undercure. DA- EPOCH-R (Petrich JCO 2014; Dunleavy Blood 2018) shows superior CR/PFS in single-arm series. Pola-R-CHP (POLARIX, Tilly NEJM 2022) approved for DLBCL — DH subgroup analysis numerically favorable. CAR-T (axi-cel, liso-cel) effective at 2L+.

Notes

ESCAT IIA. CNS prophylaxis recommended. MYC-only rearrangement (single-hit) treated as DLBCL-NOS.

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