# Linfoma maligno não-Hodgkin

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

## Identificadores

- **ORPHA**: 547 — https://www.orpha.net/en/disease/detail/547
- **OMIM**: none — https://omim.org/entry/none

## Descrição clínica

O Linfoma Não-Hodgkin (LNH) é diferente do Linfoma de Hodgkin tanto na sua aparência quanto no seu funcionamento. Uma de suas principais características é a falta das células de Reed-Sternberg. Pode aparecer em qualquer idade e geralmente se manifesta com o inchaço dos gânglios (linfonodos), seja em uma área específica ou espalhado pelo corpo, acompanhado de febre e perda de peso. A forma como a doença evolui varia de acordo com o tipo de célula envolvida. Clinicamente, o LNH é classificado como indolente (de crescimento lento), agressivo (de crescimento rápido) ou com uma evolução variável. O LNH pode ter origem em diferentes tipos de células, como as células B, T ou NK.

## Epidemiologia e herança

- **Prevalência**: 1-5 / 10 000

## Sinais e sintomas (133 fenótipos HPO)

- **Perda de peso** — HPO: HP:0001824
- **Placa eritematosa** — HPO: HP:0025474
- **Calafrios** — HPO: HP:0025143
- **Hepatoesplenomegalia** — HPO: HP:0001433
- **Pápula eritematosa** — HPO: HP:0030350
- **Anormalidade da cascata de coagulação** — HPO: HP:0003256
- **Atrofia cutânea difusa** — HPO: HP:0007488
- **Mácula eritematosa** — HPO: HP:0025475
- **Poiquilodermia** — HPO: HP:0001029
- **Alopecia** — HPO: HP:0001596
- **Úlcera cutânea** — HPO: HP:0200042
- **Pele seca** — HPO: HP:0000958
- **Eritrodermia** — HPO: HP:0001019
- **Edema** — HPO: HP:0000969
- **Morfologia anormal da pálpebra** — HPO: HP:0000492
- **Linfoma cutâneo de células T** — HPO: HP:0012192
- **Hiperpigmentação irregular** — HPO: HP:0007400
- **Manchas cutâneas hipopigmentadas** — HPO: HP:0001053
- **Morfologia anormal de linfócitos** — HPO: HP:0004332
- **Erupção cutânea** — HPO: HP:0000988
- **Morfologia anormal da unha** — HPO: HP:0001597
- **Hiperceratose** — HPO: HP:0000962
- **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
- **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
- _...e mais 93 sintomas. Ver https://raras.org/doenca/linfoma-maligno-nao-hodgkin._

## Genes associados (47)

- **HAVCR2** — Hepatitis A virus cellular receptor 2 [Disease-causing germline mutation(s) in]
  - Função: Cell surface receptor implicated in modulating innate and adaptive immune responses. Generally accepted to have an inhibiting function. Reports on stimulating functions suggest that the activity may b
- **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
- **ZBTB16** — Zinc finger and BTB domain-containing protein 16 [Candidate gene tested in]
  - Função: Acts as a transcriptional repressor (PubMed:10688654, PubMed:24359566). Transcriptional repression may be mediated through recruitment of histone deacetylases to target promoters (PubMed:10688654). Ma
- **TRD** [Candidate gene tested in]
- **STIL** — SCL-interrupting locus protein [Candidate gene tested in]
  - Função: Immediate-early gene. Plays an important role in embryonic development as well as in cellular growth and proliferation; its long-term silencing affects cell survival and cell cycle distribution as wel
- **TNFRSF1B** — Tumor necrosis factor receptor superfamily member 1B [Candidate gene tested in]
  - Função: Receptor with high affinity for TNFSF2/TNF and approximately 5-fold lower affinity for homotrimeric TNFSF1/lymphotoxin-alpha. The TRAF1/TRAF2 complex recruits the apoptotic suppressors BIRC2 and BIRC3
- **CD28** — T-cell-specific surface glycoprotein CD28 [Candidate gene tested in]
  - Função: Receptor that plays a role in T-cell activation, proliferation, survival and the maintenance of immune homeostasis (PubMed:1650475, PubMed:7568038). Functions not only as an amplifier of TCR signals b
- **CTLA4** — Cytotoxic T-lymphocyte protein 4 [Candidate gene tested in]
  - Função: Inhibitory receptor acting as a major negative regulator of T-cell responses (PubMed:11279501, PubMed:11279502, PubMed:16551244, PubMed:1714933, PubMed:18641304, PubMed:28484017). Acts as a decoy rece
- **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
- **TRA** — T cell receptor alpha chain MC.7.G5 [Candidate gene tested in]
  - Função: The alpha chain of TRAV38-2DV8*01J31*01C*01/TRBV25-1*01J2S3*01C2*01 alpha-beta T cell receptor (TR) clonotype that displays pan-cancer cell recognition via the invariant MR1 molecule. On CD8-positive

## Ensaios clínicos ativos (36)

- **NCT06137144** [RECRUITING]: AZD3470 as Monotherapy or in Combination With Anticancer Agent(s) in Participants With Haematologic Malignancies. — https://clinicaltrials.gov/study/NCT06137144
- **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
- **NCT05909059** [RECRUITING]: CAR T-cell Therapy in Patients With Renal Dysfunction — https://clinicaltrials.gov/study/NCT05909059
- **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
- **NCT05826535** [RECRUITING]: Study of Rondecabtagene Autoleucel in Aggressive Large B-Cell Lymphoma — https://clinicaltrials.gov/study/NCT05826535
- **NCT05294731** [RECRUITING]: Treatment of Chinese Participants With B-Cell Malignancies With BGB-16673, a Bruton Tyrosine Kinase-Targeted Protein-Degrader — https://clinicaltrials.gov/study/NCT05294731
- **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
- **NCT06768905** [RECRUITING]: IOMAB-CAR-T Followed by CAR-T Cell Therapy in R/R DLBCL — https://clinicaltrials.gov/study/NCT06768905
- **NCT06870760** [RECRUITING]: Pre-malignant States to Hematologic Malignancies in Firefighters — https://clinicaltrials.gov/study/NCT06870760

## Doenças relacionadas (por similaridade fenotípica)

- [Linfoma não-Hodgkin de células B](https://raras.org/doenca/linfoma-nao-hodgkin-de-celulas-b) — ORPHA:171915 — 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
- [Linfoma órgão-específico primário](https://raras.org/doenca/linfoma-orgao-especifico-primario) — ORPHA:279911 — 57 sintomas em comum
- [Linfoma cutâneo primário](https://raras.org/doenca/linfoma-cutaneo-primario) — ORPHA:542 — 56 sintomas em comum
- [Linfoma cutâneo de células T, indolente](https://raras.org/doenca/linfoma-cutaneo-de-celulas-t-indolente) — ORPHA:178548 — 54 sintomas em comum
- [Linfoma cutâneo de células T](https://raras.org/doenca/linfoma-cutaneo-de-celulas-t) — ORPHA:171901 — 54 sintomas em comum
- [Linfoma não-Hodgkin de células T](https://raras.org/doenca/linfoma-nao-hodgkin-de-celulas-t) — ORPHA:171918 — 54 sintomas em comum
- [Tumor raro induzido por vírus](https://raras.org/doenca/tumor-raro-induzido-por-virus) — ORPHA:289635 — 51 sintomas em comum
- [Macroglobulinemia de Waldenström](https://raras.org/doenca/macroglobulinemia-de-waldenstrom) — ORPHA:33226 — 48 sintomas em comum
- [Tumor relacionado com vírus Epstein-Barr](https://raras.org/doenca/tumor-relacionado-com-virus-epstein-barr) — ORPHA:289638 — 43 sintomas em comum

## Importante

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Pacientes devem consultar profissionais de saúde qualificados para decisões clínicas.

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