# Linfoma não-Hodgkin indolente de células B

> Página oficial: https://raras.org/doenca/linfoma-nao-hodgkin-indolente-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**: 300842 — https://www.orpha.net/en/disease/detail/300842
- **OMIM**: none — https://omim.org/entry/none

## Descrição clínica

Linfoma não-Hodgkin indolente de células B é um câncer de crescimento lento que afeta linfócitos B. Pode causar infecções recorrentes, sangramento gastrointestinal, problemas respiratórios e outros sintomas devido ao acúmulo de células doentes.

## Epidemiologia e herança

- **Prevalência**: Unknown

## Sinais e sintomas (73 fenótipos HPO)

- **Infecções recorrentes** — HPO: HP:0002719
- **Hemorragia gastrointestinal** — HPO: HP:0002239
- **Insuficiência respiratória** — HPO: HP:0002093
- **Proptose** — HPO: HP:0000520
- **Edema pedal** — HPO: HP:0010741
- **Anorexia** — HPO: HP:0002039
- **Aumento da concentração circulante de lactato desidrogenase** — HPO: HP:0025435
- **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
- **Linfoma** — HPO: HP:0002665
- **Polineuropatia** — HPO: HP:0001271
- **Leucemia** — HPO: HP:0001909
- **Proteinemia monoclonal de imunoglobulina M** — HPO: HP:0005508
- **Elevação policlonal de IgM** — HPO: HP:0003459
- **Transformação linfocitária prejudicada com fito-hemaglutinina** — HPO: HP:0003347
- **Imunodeficiência celular** — HPO: HP:0005374
- **Leucemia linfática crônica** — HPO: HP:0005550
- **Linfoma gástrico** — HPO: HP:0045038
- **Uveíte posterior** — HPO: HP:0012123
- **Linfadenopatia mediastinal** — HPO: HP:0100721
- **Deficiência visual** — HPO: HP:0000505
- **Morfologia anormal do sistema nasolacrimal** — HPO: HP:0000614
- **Hiperidrose** — HPO: HP:0000975
- **Dor abdominal** — HPO: HP:0002027
- **Infiltrados pulmonares** — HPO: HP:0002113
- **Constipação** — HPO: HP:0002019
- _...e mais 33 sintomas. Ver https://raras.org/doenca/linfoma-nao-hodgkin-indolente-de-celulas-b._

## Genes associados (21)

- **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
- **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
- **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
- **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
- **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
- **IGHG1** — Immunoglobulin heavy constant gamma 1 [Candidate gene tested in]
  - Função: Constant region of immunoglobulin (Ig) heavy chains. Igs are membrane-bound or secreted glycoproteins produced by B lymphocytes. In the recognition phase of humoral immunity, the membrane-bound Igs se
- **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
- **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
- **HLA-DRB1** — HLA class II histocompatibility antigen, DRB1 beta chain [Candidate gene tested in]
  - Função: A beta chain of antigen-presenting major histocompatibility complex class II (MHCII) molecule. In complex with the alpha chain HLA-DRA, displays antigenic peptides on professional antigen presenting c
- **POT1** — Protection of telomeres protein 1 [Candidate gene tested in]
  - Função: Component of the telomerase ribonucleoprotein (RNP) complex that is essential for the replication of chromosome termini. Is a component of the double-stranded telomeric DNA-binding TRF1 complex which
- **P2RX7** — P2X purinoceptor 7 [Candidate gene tested in]
  - Função: ATP-gated nonselective transmembrane cation channel that requires high millimolar concentrations of ATP for activation (PubMed:17483156, PubMed:25281740, PubMed:9038151). Upon ATP binding, it rapidly
- **BRAF** — Serine/threonine-protein kinase B-raf [Candidate gene tested in]
  - Função: Protein kinase involved in the transduction of mitogenic signals from the cell membrane to the nucleus (Probable). Phosphorylates MAP2K1, and thereby activates the MAP kinase signal transduction pathw

## Medicamentos em desenvolvimento (8)

- FLUDARABINE PHOSPHATE — Fase Phase 4 (DNA polymerase (alpha/delta/epsilon) inhibitor)
- RITUXIMAB — Fase Phase 4 (B-lymphocyte antigen CD20 binding agent)
- OFATUMUMAB — Fase Phase 4 (B-lymphocyte antigen CD20 binding agent)
- IDELALISIB — Fase Phase 4 (PI3-kinase p110-delta subunit inhibitor)
- VENETOCLAX — Fase Phase 4 (Apoptosis regulator Bcl-2 inhibitor)
- ACALABRUTINIB — Fase Phase 4 (Tyrosine-protein kinase BTK inhibitor)
- ACALABRUTINIB MALEATE — Fase Phase 4 (Tyrosine-protein kinase BTK inhibitor)
- DUVELISIB MONOHYDRATE — Fase Phase 4 (PI3-kinase p110-gamma subunit inhibitor)
- Fonte: https://platform.opentargets.org/disease/MONDO_0017594

## Ensaios clínicos ativos (18)

- **NCT06015880** [RECRUITING]: Testing the Combination of Anti-cancer Drugs Mosunetuzumab, Polatuzumab Vedotin, and Lenalidomide for the Treatment of Relapsed/Refractory Diffuse Large B-Cell Lymphoma — https://clinicaltrials.gov/study/NCT06015880
- **NCT06544265** [RECRUITING]: SynKIR-310 for Relapsed/Refractory B-NHL — https://clinicaltrials.gov/study/NCT06544265
- **NCT06784726** [RECRUITING]: Odronextamab for Relapsed and Refractory Large B-cell Lymphomas Before CAR-T — https://clinicaltrials.gov/study/NCT06784726
- **NCT06209619** [RECRUITING]: CC-99282 + Rituximab Early Post CART for Non-Hodgkin's Lymphoma — https://clinicaltrials.gov/study/NCT06209619
- **NCT05755087** [RECRUITING]: Tegavivint for Treating Patients With Relapsed or Refractory Large B-Cell Lymphoma — https://clinicaltrials.gov/study/NCT05755087
- **NCT07098364** [RECRUITING]: ST-067 in Combination With CD19-Directed CAR T-Cell Therapy (Liso-cel) in Relapsed/Refractory Large B-Cell Lymphoma — https://clinicaltrials.gov/study/NCT07098364
- **NCT06386315** [RECRUITING]: Reduced Dose Radiotherapy for the Treatment of Indolent Non-Hodgkin Lymphoma — https://clinicaltrials.gov/study/NCT06386315
- **NCT05672251** [RECRUITING]: Loncastuximab Tesirine and Mosunetuzumab for the Treatment of Relapsed or Refractory Diffuse Large B-Cell Lymphoma — https://clinicaltrials.gov/study/NCT05672251
- **NCT03571568** [RECRUITING]: A Study of BI-1206 in Combination With Rituximab With or Without Acalabrutinib in Subjects With Indolent B-Cell NHL — https://clinicaltrials.gov/study/NCT03571568
- **NCT06238648** [RECRUITING]: Epcoritamab Compared to Observation for Treating B-cell Lymphoma Patients Not in Complete Remission After CD19-directed CAR-T Therapy — https://clinicaltrials.gov/study/NCT06238648

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- [Linfoma não-Hodgkin de células B](https://raras.org/doenca/linfoma-nao-hodgkin-de-celulas-b) — ORPHA:171915 — 73 sintomas em comum
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- [Doença linfoproliferativa associada a vírus Epstein-Barr](https://raras.org/doenca/doenca-linfoproliferativa-associada-a-virus-epstein-barr) — ORPHA:289644 — 25 sintomas em comum

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