# Linfoma não-Hodgkin de células B

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

## Identificadores

- **ORPHA**: 171915 — https://www.orpha.net/en/disease/detail/171915
- **OMIM**: none — https://omim.org/entry/none

## Descrição clínica

É o tipo mais comum de linfoma não-Hodgkin. Inclui os tipos mais frequentemente encontrados, que são: linfoma difuso de grandes células B, linfoma folicular, linfoma linfocítico de pequenas células e linfoma de células B da zona marginal. -- 2003

## Epidemiologia e herança

- **Prevalência**: 1-5 / 10 000

## Sinais e sintomas (89 fenótipos HPO)

- **Obstrução intestinal** — HPO: HP:0005214
- **Derrame pericárdico** — HPO: HP:0001698
- **Insuficiência respiratória** — HPO: HP:0002093
- **Proptose** — HPO: HP:0000520
- **Edema pedal** — HPO: HP:0010741
- **Anormalidade do fígado** — HPO: HP:0001392
- **Hipercalcemia** — HPO: HP:0003072
- **Morfologia vascular retiniana anormal** — HPO: HP:0008046
- **Enxaqueca** — HPO: HP:0002076
- **Epistaxe** — HPO: HP:0000421
- **Crioglobulinemia** — HPO: HP:0100778
- **Hipercoagulabilidade** — HPO: HP:0100724
- **Vasculite** — HPO: HP:0002633
- **Hepatomegalia** — HPO: HP:0002240
- **Acidente vascular cerebral** — HPO: HP:0001297
- **Palidez** — HPO: HP:0000980
- **Comprometimento da memória** — HPO: HP:0002354
- **Edema periorbital** — HPO: HP:0100539
- **Neuropatia periférica** — HPO: HP:0009830
- **Paralisia de nervo craniano** — HPO: HP:0006824
- **Consciência/confusão reduzida** — HPO: HP:0004372
- **Taxa de sedimentação de eritrócitos elevada** — HPO: HP:0003565
- **Infecções recorrentes** — HPO: HP:0002719
- **Vertigem** — HPO: HP:0002321
- **Anormalidade dos neutrófilos** — HPO: HP:0001874
- **Distensão abdominal** — HPO: HP:0003270
- **Descargas epileptiformes multifocais** — HPO: HP:0010841
- **Anemia normocítica** — HPO: HP:0001897
- **Ataxia** — HPO: HP:0001251
- **Púrpura** — HPO: HP:0000979
- **Cutis marmorata** — HPO: HP:0000965
- **Má absorção** — HPO: HP:0002024
- **Diarreia** — HPO: HP:0002014
- **Insuficiência cardíaca congestiva** — HPO: HP:0001635
- **Urticária** — HPO: HP:0001025
- **Hemorragia retiniana** — HPO: HP:0000573
- **Deficiência auditiva** — HPO: HP:0000365
- **Sangramento gengival** — HPO: HP:0000225
- **Insuficiência renal** — HPO: HP:0000083
- **Linfoma** — HPO: HP:0002665
- _...e mais 49 sintomas. Ver https://raras.org/doenca/linfoma-nao-hodgkin-de-celulas-b._

## Genes associados (29)

- **MYD88** — Myeloid differentiation primary response protein MyD88 [Disease-causing germline mutation(s) in]
  - Função: Adapter protein involved in the Toll-like receptor and IL-1 receptor signaling pathway in the innate immune response (PubMed:15361868, PubMed:18292575, PubMed:33718825, PubMed:37971847). Acts via IRAK
- **HLF** — Hepatic leukemia factor [Candidate gene tested in]
- **MALT1** — Mucosa-associated lymphoid tissue lymphoma translocation protein 1 [Candidate gene tested in]
  - Função: Protease that enhances BCL10-induced activation: acts via formation of CBM complexes that channel adaptive and innate immune signaling downstream of CARD domain-containing proteins (CARD9, CARD11 and
- **BIRC3** — Baculoviral IAP repeat-containing protein 3 [Candidate gene tested in]
  - Função: Multi-functional protein which regulates not only caspases and apoptosis, but also modulates inflammatory signaling and immunity, mitogenic kinase signaling and cell proliferation, as well as cell inv
- **PAX5** — Paired box protein Pax-5 [Candidate gene tested in]
  - Função: Transcription factor that plays an essential role in commitment of lymphoid progenitors to the B-lymphocyte lineage (PubMed:10811620, PubMed:27181361). Fulfills a dual role by repressing B-lineage ina
- **FOXP1** — Forkhead box protein P1 [Candidate gene tested in]
  - Função: Transcriptional repressor (PubMed:18347093, PubMed:26647308). Can act with CTBP1 to synergistically repress transcription but CTPBP1 is not essential (By similarity). Plays an important role in the sp
- **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
- **ARL11** — ADP-ribosylation factor-like protein 11 [Candidate gene tested in]
  - Função: May play a role in apoptosis. May act as a tumor suppressor
- **IKZF3** — Zinc finger protein Aiolos [Candidate gene tested in]
  - Função: Transcription factor that plays an important role in the regulation of lymphocyte differentiation. Plays an essential role in regulation of B-cell differentiation, proliferation and maturation to an e
- **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
- **PBX1** — Pre-B-cell leukemia transcription factor 1 [Candidate gene tested in]
  - Função: Transcription factor which binds the DNA sequence 5'-TGATTGAT-3' as part of a heterodimer with HOX proteins such as HOXA1, HOXA5, HOXB7 and HOXB8 (PubMed:9191052). Binds to the DNA sequence 5'-TGATTGA
- **AUTS2** — Autism susceptibility gene 2 protein [Candidate gene tested in]
  - Função: Component of a Polycomb group (PcG) multiprotein PRC1-like complex, a complex class required to maintain the transcriptionally repressive state of many genes, including Hox genes, throughout developme
- **TCF3** — Transcription factor 7-like 1 [Candidate gene tested in]
  - Função: Participates in the Wnt signaling pathway. Binds to DNA and acts as a repressor in the absence of CTNNB1, and as an activator in its presence. Necessary for the terminal differentiation of epidermal c
- **RPS15** — Small ribosomal subunit protein uS19 [Candidate gene tested in]
  - Função: Component of the small ribosomal subunit (PubMed:23636399). The ribosome is a large ribonucleoprotein complex responsible for the synthesis of proteins in the cell (PubMed:23636399)
- **IGHV4-34** — Immunoglobulin heavy variable 4-34 [Candidate gene tested in]
  - Função: V region of the variable domain of immunoglobulin heavy chains that participates in the antigen recognition (PubMed:24600447). Immunoglobulins, also known as antibodies, are membrane-bound or secreted

## Ensaios clínicos ativos (37)

- **NCT06649812** [RECRUITING]: Testing the Effectiveness of a Combination Targeted Therapy (ViPOR) for Patients With Relapsed and/or Refractory Aggressive B-cell Lymphoma — https://clinicaltrials.gov/study/NCT06649812
- **NCT07215585** [RECRUITING]: AZD0486 1L Therapy for Elderly or Unfit Participants With LBCL — https://clinicaltrials.gov/study/NCT07215585
- **NCT05169515** [RECRUITING]: A Study Evaluating the Safety, Pharmacokinetics, and Efficacy of Mosunetuzumab or Glofitamab in Combination With CC-220 and/or CC-99282 in Participants With B-Cell Non-Hodgkin Lymphoma — https://clinicaltrials.gov/study/NCT05169515
- **NCT05139017** [RECRUITING]: A Study of Zilovertamab Vedotin (MK-2140) in Combination With Standard of Care in Participants With Relapsed or Refractory Diffuse Large B-Cell Lymphoma (rrDLBCL) (MK-2140-003) — https://clinicaltrials.gov/study/NCT05139017
- **NCT04609046** [RECRUITING]: Testing the Addition of Lenalidomide and Nivolumab to the Usual Treatment for Primary CNS Lymphoma — https://clinicaltrials.gov/study/NCT04609046
- **NCT06890884** [RECRUITING]: A Clinical Study of Zilovertamab Vedotin (MK-2140) Plus Rituximab Plus Cyclophosphamide, Doxorubicin, and Prednisone (R-CHP) Versus Polatuzumab Vedotin Plus R-CHP in People With Diffuse Large B-cell Lymphoma (DLBCL) (MK-2140-011/waveLINE-011) — https://clinicaltrials.gov/study/NCT06890884
- **NCT06859008** [RECRUITING]: Zanubrutinib in Combination With Sonrotoclax for the Treatment of Underrepresented Ethnic and Racial Minorities With Relapsed or Refractory B-cell Non-Hodgkin Lymphoma — https://clinicaltrials.gov/study/NCT06859008
- **NCT03223610** [RECRUITING]: Venetoclax, Ibrutinib, Prednisone, Obinutuzumab, and Revlimid (ViPOR) in Relapsed/Refractory B-cell Lymphoma — https://clinicaltrials.gov/study/NCT03223610
- **NCT04544592** [RECRUITING]: UCD19 CarT in Treatment of Pediatric B-ALL and B-NHL — https://clinicaltrials.gov/study/NCT04544592
- **NCT04094311** [RECRUITING]: Study of Out of Specification for Tisagenlecleucel — https://clinicaltrials.gov/study/NCT04094311

## Doenças relacionadas (por similaridade fenotípica)

- [Linfoma maligno não-Hodgkin](https://raras.org/doenca/linfoma-maligno-nao-hodgkin) — ORPHA:547 — 89 sintomas em comum
- [Linfoma não-Hodgkin indolente de células B](https://raras.org/doenca/linfoma-nao-hodgkin-indolente-de-celulas-b) — ORPHA:300842 — 73 sintomas em comum
- [Macroglobulinemia de Waldenström](https://raras.org/doenca/macroglobulinemia-de-waldenstrom) — ORPHA:33226 — 48 sintomas em comum
- [Tumor raro induzido por vírus](https://raras.org/doenca/tumor-raro-induzido-por-virus) — ORPHA:289635 — 44 sintomas em comum
- [Tumor relacionado com vírus Epstein-Barr](https://raras.org/doenca/tumor-relacionado-com-virus-epstein-barr) — ORPHA:289638 — 40 sintomas em comum
- [Doença linfoproliferativa associada a vírus Epstein-Barr](https://raras.org/doenca/doenca-linfoproliferativa-associada-a-virus-epstein-barr) — ORPHA:289644 — 40 sintomas em comum
- [Vasculite associada a anticorpos anti-neutrófilo citoplasmáticos (ANCA)](https://raras.org/doenca/vasculite-associada-a-anticorpos-anti-neutrofilo-citoplasmaticos-anca) — ORPHA:156152 — 30 sintomas em comum
- [Linfoma não-Hodgkin de células B agressivo](https://raras.org/doenca/linfoma-nao-hodgkin-de-celulas-b-agressivo) — ORPHA:300846 — 29 sintomas em comum
- [Hemopatia mieloide](https://raras.org/doenca/hemopatia-mieloide) — ORPHA:171895 — 29 sintomas em comum
- [Vasculite mediada por complexos imunes](https://raras.org/doenca/vasculite-mediada-por-complexos-imunes) — ORPHA:156149 — 28 sintomas em comum

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