High-Grade B-Cell Lymphoma with MYC and BCL2 and/or BCL6 rearrangements (double-hit / triple-hit)
Visão determinística da entidade fonte YAML. A autoridade clínica permanece com os IDs de fonte citados e estado de assinatura do revisor.
| ID | DIS-HGBL-DH |
|---|---|
| Tipo | Doença |
| Alegais | DH/THLLinfoma com duplo impactoHGBLHGBL-DHLinfoma de células B de alto grau, double-hit / triple-hitВисокозлоякісна B-клітинна лімфома з MYC + BCL2/BCL6 (double-hit / triple-hit) |
| Estado | reviewed 2026-04-25 | pending_clinical_signoff |
| Doenças | Nenhuma declaração |
| Fontes | 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 | 9680/3 |
|---|---|
| icd_10 | C83.3 |
| who_classification | Aggressive B-cell lymphomas — High-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements per WHO 5th ed. |
Notas
Mandatory FISH break-apart for MYC + BCL2 + BCL6 on every newly- diagnosed DLBCL — without it, double-hit is missed. Distinct from Burkitt (Ki67 ~100%, BCL2−) by Ki67 typically 70-90% + BCL2+.
Usado por
Relevância terapêutica
BMA-BCL2-REARRANGEMENT-HGBL-DH- BCL2-R como parceiro do MYC-R define HGBL-DH. A mesma gestão que a célula MYC-R: DA-EPOCH-R...BMA-MYC-REARRANGEMENT-HGBL-DH- HGBL com MYC + BCL2 (e/ou BCL6) rearranjos ('double-hit' / 'triple-hit') tem agre...
Algoritmos
ALGO-HGBL-DH-1L- ALGO-HGBL-DH-1LALGO-HGBL-DH-2L- ALGO-HGBL-DH-2L
Doença
DIS-BURKITT- Linfoma de BurkittDIS-DLBCL-NOS- Linfoma B-Cell grande difuso, NOS
Indicação
IND-HGBL-DH-1L-DAEPOCHR- IND-HGBL-DH-1L-DAEPOCHRIND-HGBL-DH-1L-RCHOP- IND-HGBL-DH-1L-RCHOPIND-HGBL-DH-2L-CART-AXICEL- IND-HGBL-DH-2L-CART-AXICEL
Questionários
QUEST-HGBL-DH-1L-STUB- Linfoma B-Cell de alta qualidade, Duplo-Hit / Triplo-Hit — primeira linha
Sinal de alerta
RF-DLBCL-HIGH-RISK-BIOLOGY- Biologia de alto risco em DLBCL NOS: duplo-expressor (MYC + BCL2 por IHC, sem rearranjo), ...RF-HGBL-DH-CNS-PROPHYLAXIS-TRIGGER- HGBL double-/triple-hit lymphoma — biology alone meets CNS-prophylaxis indication indepen...RF-HGBL-DH-EMERGENCY-TLS- High-grade B-cell lymphoma double-/triple-hit with active or imminent TLS at diagnosis: L...RF-HGBL-DH-FRAILTY-AGE- Idade > 75 OU ECOG ≥3 — toxicidade DA-EPOCH-R proibitiva; R-CHOP modificado + IT MTX profilaxi...RF-HGBL-DH-HIGH-RISK-BIOLOGY- MYC + BCL2 (and/or BCL6) rearrangement (double-/triple-hit) confirmed by FISH — DA-EPOCH-...RF-HGBL-DH-INFECTION-SCREENING- sorologia obrigatória pré-rituximab do VHB/VHC/HIV; HGBL-DH associado ao VIH recebe otimização de cART...RF-HGBL-DH-ORGAN-DYSFUNCTION- Disfunção cardíaca (LVEF < 50%) — DA-EPOCH-R (preferido para HGBL-DH/-TH) é antraciclina...RF-HGBL-DH-TRANSFORMATION-PROGRESSION- PET-2 progression (Deauville 4-5 with new lesions) or end-of-induction non-CR — HGBL-DH r...
Fontes
SRC-ESMO-DLBCL-2024- Orientação da prática clínica ESMO sobre linfoma B-Cell grande difusoSRC-ESMO-LYMPHOMA-2025- Linfomas: Orientação de Prática Clínica ESMO para o diagnóstico, tratamento e seguimento