# Linfoma não-Hodgkin de células B agressivo

> Página oficial: https://raras.org/doenca/linfoma-nao-hodgkin-de-celulas-b-agressivo
> Fonte: Raras.org — Banco de Dados de Doenças Raras em Português (CC BY-NC-SA 4.0)
> Última atualização: 2026-05-07

## Identificadores

- **ORPHA**: 300846 — https://www.orpha.net/en/disease/detail/300846
- **OMIM**: none — https://omim.org/entry/none

## Descrição clínica

Linfoma agressivo de células B, caracterizado por crescimento rápido e disseminação. Pode causar febre, perda de peso, fadiga e dispneia, associado a alterações na medula óssea e ovários. Mutações em genes como MYC e BCL2 são comuns.

## Epidemiologia e herança


## Sinais e sintomas (29 fenótipos HPO)

- **Imunodeficiência** — HPO: HP:0002721
- **Linfoma de Burkitt** — HPO: HP:0030080
- **Dispneia** — HPO: HP:0002094
- **Fadiga** — HPO: HP:0012378
- **Morfologia anormal do ovário** — HPO: HP:0000137
- **Morfologia anormal das células da medula óssea** — HPO: HP:0005561
- **Febre** — HPO: HP:0001945
- **Perda de peso** — HPO: HP:0001824
- **Linfoma de células B** — HPO: HP:0012191
- **Esplenomegalia** — HPO: HP:0001744
- **Anorexia** — HPO: HP:0002039
- **Linfadenopatia** — HPO: HP:0002716
- **Anormalidade do trato gastrointestinal** — HPO: HP:0011024
- **Neoplasia da cavidade oral** — HPO: HP:0100649
- **Proporção diminuída de células T CD4-positivas** — HPO: HP:0032218
- **Náusea e vômito** — HPO: HP:0002017
- **Morfologia anormal do pâncreas** — HPO: HP:0001732
- **Dor abdominal** — HPO: HP:0002027
- **Hemorragia gastrointestinal** — HPO: HP:0002239
- **Anormalidade do baço** — HPO: HP:0001743
- **Hiperuricemia** — HPO: HP:0002149
- **Aumento da concentração circulante de lactato desidrogenase** — HPO: HP:0025435
- **Anormalidade dos linfonodos** — HPO: HP:0002733
- **Obstrução intestinal** — HPO: HP:0005214
- **Distensão abdominal** — HPO: HP:0003270
- **Anormalidade do fígado** — HPO: HP:0001392
- **Derrame pleural** — HPO: HP:0002202
- **Morfologia anormal do peritônio** — HPO: HP:0002585
- **Derrame pericárdico** — HPO: HP:0001698

## Genes associados (8)

- **MYC** — Myc proto-oncogene protein [Disease-causing germline mutation(s) in]
  - Função: Transcription factor that binds DNA in a non-specific manner, yet also specifically recognizes the core sequence 5'-CAC[GA]TG-3' (PubMed:24940000, PubMed:25956029). Activates the transcription of grow
- **BCL6** — B-cell lymphoma 6 protein [Candidate gene tested in]
  - Função: Transcriptional repressor mainly required for germinal center (GC) formation and antibody affinity maturation which has different mechanisms of action specific to the lineage and biological functions.
- **BCL2** — Apoptosis regulator Bcl-2 [Candidate gene tested in]
  - Função: Suppresses apoptosis in a variety of cell systems including factor-dependent lymphohematopoietic and neural cells (PubMed:1508712, PubMed:8183370). Regulates cell death by controlling the mitochondria
- **XPO1** — Exportin-1 [Candidate gene tested in]
  - Função: Mediates the nuclear export of cellular proteins (cargos) bearing a leucine-rich nuclear export signal (NES) and of RNAs. In the nucleus, in association with RANBP3, binds cooperatively to the NES on
- **ALK** — ALK tyrosine kinase receptor [Candidate gene tested in]
  - Função: Neuronal receptor tyrosine kinase that is essentially and transiently expressed in specific regions of the central and peripheral nervous systems and plays an important role in the genesis and differe
- **CCND1** — G1/S-specific cyclin-D1 [Candidate gene tested in]
  - Função: Regulatory component of the cyclin D1-CDK4 (DC) complex that phosphorylates and inhibits members of the retinoblastoma (RB) protein family including RB1 and regulates the cell-cycle during G(1)/S tran
- **ATM** — Serine-protein kinase ATM [Candidate gene tested in]
  - Função: Serine/threonine protein kinase which activates checkpoint signaling upon double strand breaks (DSBs), apoptosis and genotoxic stresses such as ionizing ultraviolet A light (UVA), thereby acting as a
- **IGH** [Candidate gene tested in]

## Medicamentos em desenvolvimento (9)

- TISAGENLECLEUCEL — Fase Phase 4 (B-lymphocyte antigen CD19 binding agent)
- POLATUZUMAB VEDOTIN — Fase Phase 4 (Tubulin inhibitor)
- SELINEXOR — Fase Phase 4 (Exportin-1 inhibitor)
- LONCASTUXIMAB TESIRINE — Fase Phase 4 (B-lymphocyte antigen CD19 binding agent)
- GLOFITAMAB — Fase Phase 4 (T cell surface glycoprotein CD3 binding agent)
- EPCORITAMAB — Fase Phase 4 (T cell surface glycoprotein CD3 binding agent)
- TAFASITAMAB — Fase Phase 4 (B-lymphocyte antigen CD19 binding agent)
- AXICABTAGENE CILOLEUCEL — Fase Phase 4 (B-lymphocyte antigen CD19 binding agent)
- TEMSIROLIMUS — Fase Phase 4 (FK506-binding protein 1A inhibitor)
- Fonte: https://platform.opentargets.org/disease/MONDO_0017595

## Ensaios clínicos ativos (12)

- **NCT05826535** [RECRUITING]: Study of Rondecabtagene Autoleucel in Aggressive Large B-Cell Lymphoma — https://clinicaltrials.gov/study/NCT05826535
- **NCT05077527** [RECRUITING]: Immune Cell Therapy (CAR-T) for the Treatment of Patients With HIV and B-Cell Non-Hodgkin Lymphoma — https://clinicaltrials.gov/study/NCT05077527
- **NCT06544265** [RECRUITING]: SynKIR-310 for Relapsed/Refractory B-NHL — https://clinicaltrials.gov/study/NCT06544265
- **NCT06230224** [RECRUITING]: A Trial to Learn How Effective and Safe Odronextamab is Compared to Standard of Care for Adult Participants With Previously Treated Aggressive B-cell Non-Hodgkin Lymphoma — https://clinicaltrials.gov/study/NCT06230224
- **NCT06834373** [RECRUITING]: Golcadomide and Rituximab as Bridging Therapy for Relapsed or Refractory Aggressive B-cell Non-Hodgkin Lymphoma Before CAR T-cell Therapy — https://clinicaltrials.gov/study/NCT06834373
- **NCT07097363** [RECRUITING]: Epcoritamab With Dose Adjusted Etoposide, Cyclophosphamide, Vincristine, Doxorubicin, Prednisone and Rituximab (EPOCH-R) for the Treatment of Aggressive B-Cell Non-Hodgkin Lymphoma — https://clinicaltrials.gov/study/NCT07097363
- **NCT06534437** [RECRUITING]: MEN1703 (SEL24) to Treat Relapsed or Refractory Aggressive B-cell Non-Hodgkin Lymphoma (JASPIS-01) — https://clinicaltrials.gov/study/NCT06534437
- **NCT07059741** [RECRUITING]: Integrated Radiotherapy-optimized ASCT Sequential CAR-T Therapy for Patients With Relapsed and Refractory B-NHL — https://clinicaltrials.gov/study/NCT07059741
- **NCT03625037** [ACTIVE_NOT_RECRUITING]: First-in-Human (FIH) Trial in Patients With Relapsed, Progressive or Refractory B-Cell Lymphoma — https://clinicaltrials.gov/study/NCT03625037
- **NCT05025800** [ACTIVE_NOT_RECRUITING]: ALX148, Rituximab and Lenalidomide for the Treatment of Indolent and Aggressive B-cell Non-Hodgkin Lymphoma — https://clinicaltrials.gov/study/NCT05025800

## Doenças relacionadas (por similaridade fenotípica)

- [Tumor raro induzido por vírus](https://raras.org/doenca/tumor-raro-induzido-por-virus) — ORPHA:289635 — 29 sintomas em comum
- [Linfoma maligno não-Hodgkin](https://raras.org/doenca/linfoma-maligno-nao-hodgkin) — ORPHA:547 — 29 sintomas em comum
- [Linfoma não-Hodgkin de células B](https://raras.org/doenca/linfoma-nao-hodgkin-de-celulas-b) — ORPHA:171915 — 29 sintomas em comum
- [Doença linfoproliferativa associada a vírus Epstein-Barr](https://raras.org/doenca/doenca-linfoproliferativa-associada-a-virus-epstein-barr) — ORPHA:289644 — 28 sintomas em comum
- [Tumor relacionado com vírus Epstein-Barr](https://raras.org/doenca/tumor-relacionado-com-virus-epstein-barr) — ORPHA:289638 — 28 sintomas em comum
- [Linfoma de Burkitt](https://raras.org/doenca/linfoma-de-burkitt) — ORPHA:543 — 16 sintomas em comum
- [Lúpus eritematoso sistêmico](https://raras.org/doenca/lupus-eritematoso-sistemico) — ORPHA:536 — 14 sintomas em comum
- [Doença associada a herpesvirus humano-8](https://raras.org/doenca/doenca-associada-a-herpesvirus-humano-8) — ORPHA:102024 — 14 sintomas em comum
- [Sarcoma de tecidos moles](https://raras.org/doenca/sarcoma-de-tecidos-moles) — ORPHA:3394 — 13 sintomas em comum
- [Linfoma não-Hodgkin indolente de células B](https://raras.org/doenca/linfoma-nao-hodgkin-indolente-de-celulas-b) — ORPHA:300842 — 13 sintomas em comum

## Importante

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Pacientes devem consultar profissionais de saúde qualificados para decisões clínicas.

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**Citação sugerida**: Raras.org — Linfoma não-Hodgkin de células B agressivo. Disponível em: https://raras.org/doenca/linfoma-nao-hodgkin-de-celulas-b-agressivo
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