# Linfoma não-Hodgkin de células T

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

## Identificadores

- **ORPHA**: 171918 — https://www.orpha.net/en/disease/detail/171918
- **OMIM**: none — https://omim.org/entry/none

## Descrição clínica

Um linfoma não Hodgkin de linhagem de células T. Inclui o linfoma linfoblástico T e os linfomas de células T e NK maduras.

## Epidemiologia e herança

- **Prevalência**: 1-9 / 1 000 000

## Sinais e sintomas (54 fenótipos HPO)

- **Pápula eritematosa** — HPO: HP:0030350
- **Placa eritematosa** — HPO: HP:0025474
- **Calafrios** — HPO: HP:0025143
- **Hepatoesplenomegalia** — HPO: HP:0001433
- **Linfoma** — HPO: HP:0002665
- **Linfoma de Hodgkin** — HPO: HP:0012189
- **Pele rígida** — HPO: HP:0030053
- **Nefrocalcinose** — HPO: HP:0000121
- **Hipercalcemia** — HPO: HP:0003072
- **Cutis laxa** — HPO: HP:0000973
- **Eritema** — HPO: HP:0010783
- **Lesão renal aguda** — HPO: HP:0001919
- **Pele redundante** — HPO: HP:0001582
- **Dermatite psoriasiforme** — HPO: HP:0003765
- **Neoplasia da pele** — HPO: HP:0008069
- **Dermatite eczematoide** — HPO: HP:0000964
- **Prurido** — HPO: HP:0000989
- **Placa cutânea** — HPO: HP:0200035
- **Linfadenopatia** — HPO: HP:0002716
- **Anormalidade dos linfonodos** — HPO: HP:0002733
- **Autoimunidade** — HPO: HP:0002960
- **Edema facial** — HPO: HP:0000282
- **Hemofagocitose** — HPO: HP:0012156
- **Anemia** — HPO: HP:0001903
- **Pancitopenia** — HPO: HP:0001876
- **Esplenomegalia** — HPO: HP:0001744
- **Hipertrigliceridemia** — HPO: HP:0002155
- **Hipofibrinogenemia** — HPO: HP:0011900
- **Linfoma de células T tipo paniculite subcutânea** — HPO: HP:0034403
- **Aumento da concentração circulante de ferritina** — HPO: HP:0003281
- **Febre** — HPO: HP:0001945
- **Paniculite** — HPO: HP:0012490
- **Hiperceratose** — HPO: HP:0000962
- **Morfologia anormal da unha** — HPO: HP:0001597
- **Hepatomegalia** — HPO: HP:0002240
- **Morfologia anormal das células da medula óssea** — HPO: HP:0005561
- **Erupção cutânea** — HPO: HP:0000988
- **Morfologia anormal de linfócitos** — HPO: HP:0004332
- **Manchas cutâneas hipopigmentadas** — HPO: HP:0001053
- **Hiperpigmentação irregular** — HPO: HP:0007400
- _...e mais 14 sintomas. Ver https://raras.org/doenca/linfoma-nao-hodgkin-de-celulas-t._

## Genes associados (13)

- **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
- **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
- **JAK1** — Tyrosine-protein kinase JAK1 [Candidate gene tested in]
  - Função: Tyrosine kinase of the non-receptor type, involved in the IFN-alpha/beta/gamma signal pathway (PubMed:16239216, PubMed:28111307, PubMed:32750333, PubMed:7615558, PubMed:8232552). Kinase partner for th
- **BRCA1** — Breast cancer type 1 susceptibility protein [Candidate gene tested in]
  - Função: E3 ubiquitin-protein ligase that specifically mediates the formation of 'Lys-6'-linked polyubiquitin chains and plays a central role in DNA repair by facilitating cellular responses to DNA damage (Pub
- **NPM1** — Nucleophosmin [Candidate gene tested in]
  - Função: Involved in diverse cellular processes such as ribosome biogenesis, centrosome duplication, protein chaperoning, histone assembly, cell proliferation, and regulation of tumor suppressors p53/TP53 and
- **TP53** — Cellular tumor antigen p53 [Candidate gene tested in]
  - Função: Multifunctional transcription factor that induces cell cycle arrest, DNA repair or apoptosis upon binding to its target DNA sequence (PubMed:11025664, PubMed:12524540, PubMed:12810724, PubMed:15186775
- **BRCA2** — Breast cancer type 2 susceptibility protein [Candidate gene tested in]
  - Função: Involved in double-strand break repair and/or homologous recombination. Binds RAD51 and potentiates recombinational DNA repair by promoting assembly of RAD51 onto single-stranded DNA (ssDNA). Acts by
- **TYK2** — Non-receptor tyrosine-protein kinase TYK2 [Candidate gene tested in]
  - Função: Tyrosine kinase of the non-receptor type involved in numerous cytokines and interferons signaling, which regulates cell growth, development, cell migration, innate and adaptive immunity (PubMed:105422
- **JAK2** — Tyrosine-protein kinase JAK2 [Candidate gene tested in]
  - Função: Non-receptor tyrosine kinase involved in various processes such as cell growth, development, differentiation or histone modifications. Mediates essential signaling events in both innate and adaptive i
- **STAT3** — Signal transducer and activator of transcription 3 [Candidate gene tested in]
  - Função: Signal transducer and transcription activator that mediates cellular responses to interleukins, KITLG/SCF, LEP and other growth factors (PubMed:10688651, PubMed:12359225, PubMed:12873986, PubMed:15194

## Medicamentos em desenvolvimento (10)

- BRENTUXIMAB VEDOTIN — Fase Phase 4 (Tubulin inhibitor)
- DEXAMETHASONE SODIUM PHOSPHATE — Fase Phase 4 (Glucocorticoid receptor agonist)
- PREDNISOLONE — Fase Phase 4 (Glucocorticoid receptor agonist)
- MOGAMULIZUMAB — Fase Phase 4 (C-C chemokine receptor type 4 cross-linking agent)
- METHOTREXATE — Fase Phase 4 (Dihydrofolate reductase inhibitor)
- DEXAMETHASONE — Fase Phase 4 (Glucocorticoid receptor agonist)
- PREDNISONE — Fase Phase 4 (Glucocorticoid receptor agonist)
- BEXAROTENE — Fase Phase 4 (Retinoid X receptor agonist)
- PRALATREXATE — Fase Phase 4 (Dihydrofolate reductase inhibitor)
- ROMIDEPSIN — Fase Phase 4 (Histone deacetylase inhibitor)
- Fonte: https://platform.opentargets.org/disease/MONDO_0015760

## Ensaios clínicos ativos (38)

- **NCT06137144** [RECRUITING]: AZD3470 as Monotherapy or in Combination With Anticancer Agent(s) in Participants With Haematologic Malignancies. — https://clinicaltrials.gov/study/NCT06137144
- **NCT04984837** [RECRUITING]: Study of Lacutamab in Peripheral T-cell Lymphoma — https://clinicaltrials.gov/study/NCT04984837
- **NCT05475925** [RECRUITING]: A Study of DR-01 in Subjects With Large Granular Lymphocytic Leukemia or Cytotoxic Lymphomas — https://clinicaltrials.gov/study/NCT05475925
- **NCT07496229** [RECRUITING]: Assessing Treatment of T-Cell Lymphoma With GW5282 in Combination With Golidocitinib (BEI-DOU3) — https://clinicaltrials.gov/study/NCT07496229
- **NCT07356245** [RECRUITING]: Ruxolitinib Maintenance Post-Hematopoietic Stem Cell Transplant T-Cell Lymphoma — https://clinicaltrials.gov/study/NCT07356245
- **NCT06583083** [RECRUITING]: Sintilimab With P-GEMOX Versus the P-GEMOX in the Teatment of Advanced-stage Extranodal Natural Killer/T Cell Lymphoma — https://clinicaltrials.gov/study/NCT06583083
- **NCT06698822** [RECRUITING]: A Phase 2 Trial to Assess Safety and Efficacy of Tofacitinib 2% Cream in the Treatment of Cutaneous T-cell Lymphoma (CTCL), Stages IA, IB, and IIA — https://clinicaltrials.gov/study/NCT06698822
- **NCT05996185** [RECRUITING]: Study of Mogamulizumab With DA-EPOCH or CHOEP in Patients With Aggressive T-cell Lymphoma — https://clinicaltrials.gov/study/NCT05996185
- **NCT06176690** [RECRUITING]: Constitutive IL7R (C7R) Modified Banked Allogeneic CD30.CAR EBVSTS for CD30-Positive Lymphomas — https://clinicaltrials.gov/study/NCT06176690
- **NCT03017820** [RECRUITING]: A Vaccine (VSV-hIFNβ-NIS) With or Without Cyclophosphamide and Combinations of Ipilimumab, Nivolumab, and Cemiplimab in Treating Relapsed or Refractory Multiple Myeloma, Acute Myeloid Leukemia or Lymphoma — https://clinicaltrials.gov/study/NCT03017820

## Doenças relacionadas (por similaridade fenotípica)

- [Linfoma cutâneo primário](https://raras.org/doenca/linfoma-cutaneo-primario) — ORPHA:542 — 54 sintomas em comum
- [Linfoma maligno não-Hodgkin](https://raras.org/doenca/linfoma-maligno-nao-hodgkin) — ORPHA:547 — 54 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 órgão-específico primário](https://raras.org/doenca/linfoma-orgao-especifico-primario) — ORPHA:279911 — 54 sintomas em comum
- [Micose fungoide e variantes](https://raras.org/doenca/micose-fungoide-e-variantes) — ORPHA:178566 — 38 sintomas em comum
- [Micose fungoide clássica](https://raras.org/doenca/micose-fungoide-classica) — ORPHA:2584 — 30 sintomas em comum
- [Linfoma de células T, paniculite subcutânea-like](https://raras.org/doenca/linfoma-de-celulas-t-paniculite-subcutanea-like) — ORPHA:86884 — 20 sintomas em comum
- [Mastocitose](https://raras.org/doenca/mastocitose) — ORPHA:98292 — 18 sintomas em comum
- [Síndrome Omenn](https://raras.org/doenca/sindrome-omenn) — ORPHA:39041 — 18 sintomas em comum

## Importante

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Pacientes devem consultar profissionais de saúde qualificados para decisões clínicas.

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