Raras
Buscar doenças, sintomas, genes...
Linfoma não-Hodgkin de células T
ORPHA:171918DOENÇA RARA

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

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

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

Pesquisas ativas
38 ensaios
1250 total registrados no ClinicalTrials.gov
Publicações científicas
239 artigos
Último publicado: 2026
Medicamentos
10 registrados
BRENTUXIMAB VEDOTIN, DEXAMETHASONE SODIUM PHOSPHATE, PREDNISOLONE

Tem tratamento?

10 medicamentos registrados
Ver detalhes, fases e interações →
BRENTUXIMAB VEDOTINDEXAMETHASONE SODIUM PHOSPHATEPREDNISOLONEMOGAMULIZUMABMETHOTREXATEDEXAMETHASONEPREDNISONEBEXAROTENEPRALATREXATEROMIDEPSIN

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.99
Europe
🏥
SUS: Sem cobertura SUSScore: 0%
Você se identifica com essa condição?
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🧬
Pele e cabelo
17 sintomas
🩸
Sangue
8 sintomas
🫃
Digestivo
3 sintomas
🫘
Rins
2 sintomas
🛡️
Imunológico
1 sintomas
😀
Face
1 sintomas

+ 19 sintomas em outras categorias

Características mais comuns

Pápula eritematosa
Placa eritematosa
Calafrios
Hepatoesplenomegalia
Linfoma
Linfoma de Hodgkin
54sintomas
Sem dados (54)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 54 características clínicas mais associadas, ordenadas por frequência.

Pápula eritematosaErythematous papule
Placa eritematosaErythematous plaque
CalafriosChills
HepatoesplenomegaliaHepatosplenomegaly
LinfomaLymphoma

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico239PubMed
Últimos 10 anos133publicações
Pico202317 papers
Linha do tempo
2026Hoje · 2026🧪 1988Primeiro ensaio clínico📈 2023Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

13 genes identificados com associação a esta condição.

HAVCR2Hepatitis A virus cellular receptor 2Disease-causing germline mutation(s) inTolerante
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 be influenced by the cellular context and/or the respective ligand (PubMed:24825777). Regulates macrophage activation (PubMed:11823861). Inhibits T-helper type 1 lymphocyte (Th1)-mediated auto- and alloimmune responses and promotes immunological tolerance (PubMed:14556005). In CD8+ cells attenuates T

LOCALIZAÇÃO

Cell membraneCell junction

VIAS BIOLÓGICAS (1)
Interleukin-2 family signaling
EXPRESSÃO TECIDUAL(Ubíquo)
Pulmão
25.9 TPM
Baço
24.8 TPM
Sangue
18.0 TPM
Brain Spinal cord cervical c-1
14.4 TPM
Rim - Córtex
11.5 TPM
OUTRAS DOENÇAS (1)
subcutaneous panniculitis-like T-cell lymphoma
HGNC:18437UniProt:Q8TDQ0
ALKALK tyrosine kinase receptorCandidate gene tested inRestrito
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 differentiation of the nervous system (PubMed:11121404, PubMed:11387242, PubMed:16317043, PubMed:17274988, PubMed:30061385, PubMed:34646012, PubMed:34819673). Also acts as a key thinness protein involved in the resistance to weight gain: in hypothalamic neurons, controls energy expenditure acting as a nega

LOCALIZAÇÃO

Cell membrane

VIAS BIOLÓGICAS (2)
Signaling by ALKMDK and PTN in ALK signaling
OUTRAS DOENÇAS (9)
large congenital melanocytic nevusneuroblastomaganglioneuroblastomadifferentiated thyroid carcinoma
HGNC:427UniProt:Q9UM73
TNFRSF1BTumor necrosis factor receptor superfamily member 1BCandidate gene tested inModerado
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 to TNFRSF1B/TNFR2. This receptor mediates most of the metabolic effects of TNF. Isoform 2 blocks TNF-induced apoptosis, which suggests that it regulates TNF function by antagonizing its biological activity

LOCALIZAÇÃO

Cell membraneSecreted

VIAS BIOLÓGICAS (4)
Neutrophil degranulationTNFR2 non-canonical NF-kB pathwayTNFs bind their physiological receptorsInterleukin-4 and Interleukin-13 signaling
EXPRESSÃO TECIDUAL(Ubíquo)
Sangue
474.3 TPM
Baço
180.8 TPM
Tecido adiposo
110.3 TPM
Linfócitos
105.9 TPM
Pulmão
100.4 TPM
OUTRAS DOENÇAS (2)
mycosis fungoidesSezary syndrome
HGNC:11917UniProt:P20333
CD28T-cell-specific surface glycoprotein CD28Candidate gene tested inRestrito
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 but delivers unique signals that control intracellular biochemical events that alter the gene expression program of T-cells (PubMed:24665965). Stimulation upon engagement of its cognate ligands CD80 or CD86 increases proliferation and expression of various cytokines in particular IL2 production in bo

LOCALIZAÇÃO

Cell membraneCell surface

VIAS BIOLÓGICAS (2)
Co-stimulation by CD28Nef mediated downregulation of CD28 cell surface expression
MECANISMO DE DOENÇA

Immunodeficiency 123 with HPV-related verrucosis

An autosomal recessive immunologic disorder characterized by susceptibility to human papilloma virus (HPV) infections and the development of HPV-related common verrucosis in the first decade of life. In some patients with HPV2 infection, warts may progress to severe generalized hyperkeratotic cutaneous papillomatosis with cutaneous horns ('tree-man' phenotype). In patients with HPV4 infection, warts remains stable and may even regress with age.

OUTRAS DOENÇAS (3)
immunodeficiency 123 with HPV-related verrucosismycosis fungoidesSezary syndrome
HGNC:1653UniProt:P10747
CTLA4Cytotoxic T-lymphocyte protein 4Candidate gene tested inAltamente restrito
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 receptor: the affinity of CTLA4 for its natural B7 family ligands, CD80 and CD86, is considerably stronger than the affinity of their cognate stimulatory coreceptor CD28 (PubMed:11279501, PubMed:11279502, PubMed:16551244, PubMed:1714933, PubMed:28484017)

LOCALIZAÇÃO

Cell membrane

VIAS BIOLÓGICAS (3)
Co-stimulation by CD28Co-inhibition by CTLA4RUNX1 and FOXP3 control the development of regulatory T lymphocytes (Tregs)
MECANISMO DE DOENÇA

Systemic lupus erythematosus

A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.

EXPRESSÃO TECIDUAL(Tecido-específico)
Baço
5.8 TPM
Intestino delgado
4.9 TPM
Pulmão
4.3 TPM
Testículo
2.5 TPM
Sangue
1.8 TPM
OUTRAS DOENÇAS (6)
autoimmune lymphoproliferative syndrome due to CTLA4 haploinsufficiencysystemic lupus erythematosusgranulomatosis with polyangiitismycosis fungoides
HGNC:2505UniProt:P16410
JAK1Tyrosine-protein kinase JAK1Candidate gene tested inAltamente restrito
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 the interleukin (IL)-2 receptor (PubMed:11909529) as well as interleukin (IL)-10 receptor (PubMed:12133952). Kinase partner for the type I interferon receptor IFNAR2 (PubMed:16239216, PubMed:28111307, PubMed:32750333, PubMed:7615558, PubMed:8232552). In response to interferon-binding to IFNAR1-IFNAR2

LOCALIZAÇÃO

Endomembrane system

VIAS BIOLÓGICAS (10)
Interleukin-2 signalingInterleukin-15 signalingInterferon gamma signalingRegulation of IFNG signalingPotential therapeutics for SARS
MECANISMO DE DOENÇA

Autoinflammation, immune dysregulation, and eosinophilia

An autosomal dominant disorder characterized by immune dysregulation, severe atopic dermatitis, and chronic gastrointestinal inflammation. Additional features include asthma, food or environmental allergies, as well as poor overall growth with short stature.

EXPRESSÃO TECIDUAL(Ubíquo)
Fibroblastos
139.9 TPM
Tecido adiposo
124.0 TPM
Pulmão
110.0 TPM
Cervix Endocervix
107.3 TPM
Ovário
101.4 TPM
OUTRAS DOENÇAS (3)
autoinflammation, immune dysregulation, and eosinophiliabreast implant-associated anaplastic large cell lymphomaautosomal recessive mendelian susceptibility to mycobacterial diseases due to partial jak1 deficiency
HGNC:6190UniProt:P23458
BRCA1Breast cancer type 1 susceptibility proteinCandidate gene tested inTolerante
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 (PubMed:10500182, PubMed:12887909, PubMed:12890688, PubMed:14976165, PubMed:16818604, PubMed:17525340, PubMed:19261748). It is unclear whether it also mediates the formation of other types of polyubiquitin chains (PubMed:12890688). The BRCA1-BARD1 heterodimer coordinates a diverse range of cellular path

LOCALIZAÇÃO

NucleusChromosomeCytoplasm

VIAS BIOLÓGICAS (10)
Recruitment and ATM-mediated phosphorylation of repair and signaling proteins at DNA double strand breaksMetalloprotease DUBsTP53 Regulates Transcription of DNA Repair GenesTranscriptional Regulation by E2F6Meiotic synapsis
MECANISMO DE DOENÇA

Breast cancer

A common malignancy originating from breast epithelial tissue. Breast neoplasms can be distinguished by their histologic pattern. Invasive ductal carcinoma is by far the most common type. Breast cancer is etiologically and genetically heterogeneous. Important genetic factors have been indicated by familial occurrence and bilateral involvement. Mutations at more than one locus can be involved in different families or even in the same case.

OUTRAS DOENÇAS (12)
Fanconi anemia, complementation group Sbreast-ovarian cancer, familial, susceptibility to, 1BRCA1-related cancer predispositionprostate cancer, hereditary
HGNC:1100UniProt:P38398
NPM1NucleophosminCandidate gene tested inAltamente restrito
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 ARF. Binds ribosome presumably to drive ribosome nuclear export. Associated with nucleolar ribonucleoprotein structures and bind single-stranded nucleic acids. Acts as a chaperonin for the core histones H3, H2B and H4. Stimulates APEX1 endonuclease activity on apurinic/apyrimidinic (AP) double-stran

LOCALIZAÇÃO

Nucleus, nucleolusNucleus, nucleoplasmCytoplasm, cytoskeleton, microtubule organizing center, centrosome

VIAS BIOLÓGICAS (4)
PKR-mediated signalingTP53 regulates transcription of additional cell cycle genes whose exact role in the p53 pathway remain uncertainNuclear import of Rev proteinNuclear events stimulated by ALK signaling in cancer
EXPRESSÃO TECIDUAL(Ubíquo)
Linfócitos
1517.5 TPM
Fibroblastos
1222.0 TPM
Ovário
1031.3 TPM
Cervix Endocervix
640.5 TPM
Útero
622.0 TPM
OUTRAS DOENÇAS (8)
acute myeloid leukemiaacute promyelocytic leukemiaacute myeloid leukemia with NPM1 somatic mutationsdyskeratosis congenita
HGNC:7910UniProt:P06748
TP53Cellular tumor antigen p53Candidate gene tested inAltamente restrito
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, PubMed:15340061, PubMed:17317671, PubMed:17349958, PubMed:19556538, PubMed:20673990, PubMed:20959462, PubMed:22726440, PubMed:24051492, PubMed:24652652, PubMed:35618207, PubMed:36634798, PubMed:38653238, PubMed:9840937). Acts as a tumor suppressor in many tumor types; induces growth arrest or apop

LOCALIZAÇÃO

CytoplasmNucleusNucleus, PML bodyEndoplasmic reticulumMitochondrion matrixCytoplasm, cytoskeleton, microtubule organizing center, centrosome

VIAS BIOLÓGICAS (10)
TP53 Regulates Metabolic GenesRegulation of TP53 ExpressionRegulation of TP53 DegradationOncogene Induced SenescenceOxidative Stress Induced Senescence
EXPRESSÃO TECIDUAL(Ubíquo)
Linfócitos
73.0 TPM
Skin Sun Exposed Lower leg
37.0 TPM
Skin Not Sun Exposed Suprapubic
35.2 TPM
Fibroblastos
32.9 TPM
Ovário
32.4 TPM
OUTRAS DOENÇAS (29)
Li-Fraumeni syndromenasopharyngeal carcinoma, susceptibility to, 1hepatocellular carcinomafamilial pancreatic carcinoma
HGNC:11998UniProt:P04637
BRCA2Breast cancer type 2 susceptibility proteinCandidate gene tested inTolerante
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 targeting RAD51 to ssDNA over double-stranded DNA, enabling RAD51 to displace replication protein-A (RPA) from ssDNA and stabilizing RAD51-ssDNA filaments by blocking ATP hydrolysis. Part of a PALB2-scaffolded HR complex containing RAD51C and which is thought to play a role in DNA repair by HR. May

LOCALIZAÇÃO

NucleusCytoplasm, cytoskeleton, microtubule organizing center, centrosome

VIAS BIOLÓGICAS (3)
Presynaptic phase of homologous DNA pairing and strand exchangeHDR through MMEJ (alt-NHEJ)Meiotic recombination
MECANISMO DE DOENÇA

Breast cancer

A common malignancy originating from breast epithelial tissue. Breast neoplasms can be distinguished by their histologic pattern. Invasive ductal carcinoma is by far the most common type. Breast cancer is etiologically and genetically heterogeneous. Important genetic factors have been indicated by familial occurrence and bilateral involvement. Mutations at more than one locus can be involved in different families or even in the same case.

OUTRAS DOENÇAS (17)
Wilms tumor 1Fanconi anemia complementation group D1breast-ovarian cancer, familial, susceptibility to, 2BRCA2-related cancer predisposition
HGNC:1101UniProt:P51587
TYK2Non-receptor tyrosine-protein kinase TYK2Candidate gene tested inTolerante
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:10542297, PubMed:10995743, PubMed:7657660, PubMed:7813427, PubMed:8232552). Plays both structural and catalytic roles in numerous interleukins and interferons (IFN-alpha/beta) signaling (PubMed:10542297). Associates with heterodimeric cytokine receptor complexes and activates STAT family members including

LOCALIZAÇÃO

VIAS BIOLÓGICAS (10)
Interferon alpha/beta signalingRegulation of IFNA/IFNB signalingPotential therapeutics for SARSInterleukin-20 family signalingOther interleukin signaling
MECANISMO DE DOENÇA

Immunodeficiency 35

A primary immunodeficiency characterized by recurrent skin abscesses, pneumonia, and highly elevated serum IgE.

EXPRESSÃO TECIDUAL(Ubíquo)
Linfócitos
100.5 TPM
Baço
95.0 TPM
Sangue
78.6 TPM
Pulmão
65.9 TPM
Nervo tibial
65.4 TPM
OUTRAS DOENÇAS (3)
immunodeficiency 35primary cutaneous anaplastic large cell lymphomalymphomatoid papulosis
HGNC:12440UniProt:P29597
JAK2Tyrosine-protein kinase JAK2Candidate gene tested inTolerante
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 immunity. In the cytoplasm, plays a pivotal role in signal transduction via its association with type I receptors such as growth hormone (GHR), prolactin (PRLR), leptin (LEPR), erythropoietin (EPOR), thrombopoietin receptor (MPL/TPOR); or type II receptors including IFN-alpha, IFN-beta, IFN-gamma and

LOCALIZAÇÃO

Endomembrane systemCytoplasmNucleus

VIAS BIOLÓGICAS (10)
Interleukin-20 family signalingRAF activationSignaling downstream of RAS mutantsSignaling by moderate kinase activity BRAF mutantsParadoxical activation of RAF signaling by kinase inactive BRAF
EXPRESSÃO TECIDUAL(Ubíquo)
Artéria tibial
68.9 TPM
Aorta
46.9 TPM
Artéria coronária
31.6 TPM
Tecido adiposo
20.8 TPM
Nervo tibial
20.0 TPM
OUTRAS DOENÇAS (9)
thrombocythemia 3acquired polycythemia veraprimary myelofibrosisprimary familial polycythemia due to EPO receptor mutation
HGNC:6192UniProt:O60674
STAT3Signal transducer and activator of transcription 3Candidate gene tested inAltamente restrito
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:15194700, PubMed:15653507, PubMed:16285960, PubMed:17344214, PubMed:18242580, PubMed:18782771, PubMed:22306293, PubMed:23084476, PubMed:28262505, PubMed:32929201, PubMed:38404237). Once activated, recruits coactivators, such as NCOA1 or MED1, to the promoter region of the target gene (PubMed:15653507, Pu

LOCALIZAÇÃO

CytoplasmNucleus

VIAS BIOLÓGICAS (10)
Interleukin-20 family signalingDownstream signal transductionInterleukin-15 signalingSignaling by SCF-KITInterleukin-9 signaling
MECANISMO DE DOENÇA

Hyper-IgE syndrome 1, autosomal dominant, with recurrent infections

A rare disorder of immunity and connective tissue characterized by immunodeficiency, chronic eosinophilia, distinctive coarse facial appearance, abnormal dentition, hyperextensibility of the joints, and bone fractures.

EXPRESSÃO TECIDUAL(Ubíquo)
Pulmão
173.0 TPM
Artéria tibial
145.7 TPM
Aorta
144.8 TPM
Adipose Visceral Omentum
140.2 TPM
Fallopian Tube
136.9 TPM
OUTRAS DOENÇAS (7)
hyper-IgE recurrent infection syndrome 1, autosomal dominantSTAT3-related early-onset multisystem autoimmune diseasebreast implant-associated anaplastic large cell lymphomaacute promyelocytic leukemia
HGNC:11364UniProt:P40763

Medicamentos e terapias

BRENTUXIMAB VEDOTINPhase 4

Mecanismo: Tubulin inhibitor

DEXAMETHASONE SODIUM PHOSPHATEPhase 4

Mecanismo: Glucocorticoid receptor agonist

PREDNISOLONEPhase 4

Mecanismo: Glucocorticoid receptor agonist

MOGAMULIZUMABPhase 4

Mecanismo: C-C chemokine receptor type 4 cross-linking agent

METHOTREXATEPhase 4

Mecanismo: Dihydrofolate reductase inhibitor

DEXAMETHASONEPhase 4

Mecanismo: Glucocorticoid receptor agonist

PREDNISONEPhase 4

Mecanismo: Glucocorticoid receptor agonist

BEXAROTENEPhase 4

Mecanismo: Retinoid X receptor agonist

PRALATREXATEPhase 4

Mecanismo: Dihydrofolate reductase inhibitor

ROMIDEPSINPhase 4

Mecanismo: Histone deacetylase inhibitor

Ver mais no OpenTargets

Variantes genéticas (ClinVar)

4,925 variantes patogênicas registradas no ClinVar.

🧬 HAVCR2: GRCh37/hg19 5q33.2-34(chr5:154849998-164037131)x1 ()
🧬 HAVCR2: NM_032782.5(HAVCR2):c.833A>G (p.Asn278Ser) ()
🧬 HAVCR2: NM_032782.5(HAVCR2):c.319A>T (p.Ile107Phe) ()
🧬 HAVCR2: NC_000005.9:g.(?_155338082)_(156899968_?)del ()
🧬 HAVCR2: GRCh37/hg19 5q33.1-35.2(chr5:150535183-172906793)x3 ()
Ver todas no ClinVar

Vias biológicas (Reactome)

150 vias biológicas associadas aos genes desta condição.

Interleukin-2 family signaling Signaling by ALK ALK mutants bind TKIs ASP-3026-resistant ALK mutants NVP-TAE684-resistant ALK mutants alectinib-resistant ALK mutants brigatinib-resistant ALK mutants ceritinib-resistant ALK mutants crizotinib-resistant ALK mutants lorlatinib-resistant ALK mutants Signaling by ALK fusions and activated point mutants MDK and PTN in ALK signaling TNFR2 non-canonical NF-kB pathway TNFs bind their physiological receptors Interleukin-10 signaling Interleukin-4 and Interleukin-13 signaling Neutrophil degranulation PIP3 activates AKT signaling Nef mediated downregulation of CD28 cell surface expression Constitutive Signaling by Aberrant PI3K in Cancer Co-stimulation by CD28 CD28 dependent PI3K/Akt signaling CD28 dependent Vav1 pathway PI5P, PP2A and IER3 Regulate PI3K/AKT Signaling Co-inhibition by CTLA4 RUNX1 and FOXP3 control the development of regulatory T lymphocytes (Tregs) Interleukin-6 signaling MAPK3 (ERK1) activation MAPK1 (ERK2) activation ISG15 antiviral mechanism Interleukin-7 signaling Other interleukin signaling RAF/MAP kinase cascade IL-6-type cytokine receptor ligand interactions Interferon gamma signaling Regulation of IFNG signaling Interleukin-20 family signaling Interleukin-15 signaling Interleukin-35 Signalling Interleukin-9 signaling Interleukin-2 signaling Interleukin-12 signaling Interleukin-27 signaling Interleukin-21 signaling Interferon alpha/beta signaling Interleukin receptor SHC signaling Regulation of IFNA/IFNB signaling Signaling by CSF3 (G-CSF) Potential therapeutics for SARS Inactivation of CSF3 (G-CSF) signaling SARS-CoV-2 activates/modulates innate and adaptive immune responses IFNG signaling activates MAPKs Evasion by RSV of host interferon responses PD-L1(CD274) glycosylation and translocation to plasma membrane Meiotic synapsis SUMOylation of DNA damage response and repair proteins HDR through Single Strand Annealing (SSA) HDR through Homologous Recombination (HRR) Metalloprotease DUBs Resolution of D-loop Structures through Synthesis-Dependent Strand Annealing (SDSA) Recruitment and ATM-mediated phosphorylation of repair and signaling proteins at DNA double strand breaks Resolution of D-loop Structures through Holliday Junction Intermediates Nonhomologous End-Joining (NHEJ) Homologous DNA Pairing and Strand Exchange Processing of DNA double-strand break ends Presynaptic phase of homologous DNA pairing and strand exchange TP53 Regulates Transcription of DNA Repair Genes Regulation of TP53 Activity through Phosphorylation G2/M DNA damage checkpoint Neddylation Transcriptional Regulation by E2F6 Meiotic recombination Defective DNA double strand break response due to BRCA1 loss of function Defective DNA double strand break response due to BARD1 loss of function Defective homologous recombination repair (HRR) due to BRCA1 loss of function Defective HDR through Homologous Recombination Repair (HRR) due to PALB2 loss of BRCA1 binding function Defective HDR through Homologous Recombination Repair (HRR) due to PALB2 loss of BRCA2/RAD51/RAD51C binding function Impaired BRCA2 binding to RAD51 Impaired BRCA2 binding to PALB2 KEAP1-NFE2L2 pathway Regulation of MITF-M-dependent genes involved in DNA replication, damage repair and senescence Nuclear import of Rev protein SUMOylation of transcription cofactors Deposition of new CENPA-containing nucleosomes at the centromere TP53 regulates transcription of additional cell cycle genes whose exact role in the p53 pathway remain uncertain TFAP2A acts as a transcriptional repressor during retinoic acid induced cell differentiation SARS-CoV-1-host interactions Nuclear events stimulated by ALK signaling in cancer PKR-mediated signaling Activation of NOXA and translocation to mitochondria Activation of PUMA and translocation to mitochondria Pre-NOTCH Transcription and Translation Oxidative Stress Induced Senescence Formation of Senescence-Associated Heterochromatin Foci (SAHF) Oncogene Induced Senescence DNA Damage/Telomere Stress Induced Senescence SUMOylation of transcription factors Autodegradation of the E3 ubiquitin ligase COP1 Association of TriC/CCT with target proteins during biosynthesis Pyroptosis TP53 Regulates Metabolic Genes Ub-specific processing proteases Ovarian tumor domain proteases TP53 Regulates Transcription of Genes Involved in Cytochrome C Release TP53 regulates transcription of several additional cell death genes whose specific roles in p53-dependent apoptosis remain uncertain TP53 Regulates Transcription of Caspase Activators and Caspases TP53 Regulates Transcription of Death Receptors and Ligands TP53 Regulates Transcription of Genes Involved in G2 Cell Cycle Arrest TP53 Regulates Transcription of Genes Involved in G1 Cell Cycle Arrest Regulation of TP53 Expression Regulation of TP53 Degradation Regulation of TP53 Activity through Acetylation Regulation of TP53 Activity through Association with Co-factors Regulation of TP53 Activity through Methylation HDR through MMEJ (alt-NHEJ) Impaired BRCA2 translocation to the nucleus Impaired BRCA2 binding to SEM1 (DSS1) Interferon Signaling Interleukin-23 signaling Interleukin-12 family signaling Prolactin receptor signaling Signaling by SCF-KIT Signaling by Leptin RMTs methylate histone arginines Interleukin-3, Interleukin-5 and GM-CSF signaling RAF activation Signaling by moderate kinase activity BRAF mutants Signaling by BRAF and RAF1 fusions Paradoxical activation of RAF signaling by kinase inactive BRAF Cyclin D associated events in G1 Signaling by Erythropoietin Erythropoietin activates Phosphoinositide-3-kinase (PI3K) Erythropoietin activates Phospholipase C gamma (PLCG) Erythropoietin activates STAT5 Erythropoietin activates RAS Signaling downstream of RAS mutants BH3-only proteins associate with and inactivate anti-apoptotic BCL-2 members Signaling by cytosolic FGFR1 fusion mutants Downstream signal transduction Signalling to STAT3 Senescence-Associated Secretory Phenotype (SASP) POU5F1 (OCT4), SOX2, NANOG activate genes related to proliferation Transcriptional regulation of pluripotent stem cells PTK6 Activates STAT3 MET activates STAT3 Interleukin-37 signaling Transcriptional regulation of granulopoiesis Signaling by phosphorylated juxtamembrane, extracellular and kinase domain KIT mutants Signaling by PDGFRA transmembrane, juxtamembrane and kinase domain mutants Signaling by PDGFRA extracellular domain mutants

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado10
3Fase 32
2Fase 212
1Fase 15
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Medicamentos catalogadosEnsaios clínicos· 10 medicamentos · 20 ensaios
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Publicações mais relevantes

Timeline de publicações
136 papers (10 anos)
#1

Incidence of Hypertension During Induction Therapy in Children with Acute Lymphoblastic Leukemia.

Indian pediatrics2026 Jan

To estimate the incidence of hypertension during induction therapy in children with acute lymphoblastic leukemia (ALL), comparing a systematically monitored prospective cohort to a retrospective cohort lacking regular blood pressure monitoring. This ambispective observational study was conducted at a pediatric hematology-oncology unit in Chandigarh, India. The prospective cohort included 46 children with B-/T-ALL or T-cell non-Hodgkin lymphoma enrolled between September 2023 and August 2024, whose blood pressure was monitored weekly during induction therapy using the auscultatory method with an aneroid sphygmomanometer. Hypertension was defined according to the 2017 American Academy of Pediatrics guidelines. The retrospective cohort comprised 160 children diagnosed between June 2022 and July 2023, whose case files were reviewed for documented hypertension or posterior reversible encephalopathy syndrome (PRES). In the retrospective cohort, blood pressure was not monitored systematically and was detected incidentally during routine care or at the time of admission for intercurrent illnesses. Hypertension was observed in 27 (58.7%) patients in the prospective cohort, compared to 20 (12.5%) in the retrospective (P < 0.001). Most (92.6%) cases of hypertension in the prospective cohort occurred within the first two weeks of induction. No case vs. two (1.3%) cases of PRES were reported in the prospective and retrospective cohorts. The incidence of hypertension during induction therapy in childhood ALL was 58.7%, with most cases occurring within the first two weeks. Incorporating blood pressure monitoring into routine care is essential for children with ALL receiving induction chemotherapy.

#2

[Research Progress in ALK+/- Anaplastic Large Cell Lymphoma--Review].

Zhongguo shi yan xue ye xue za zhi2026 Feb

Anaplastic large cell lymphoma (ALCL) is a rare CD30-positive T-cell non-Hodgkin lymphoma, primarily classified into two subtypes: ALK-positive ( ALK +) and ALK-negative ( ALK -). ALK + ALCL is commonly seen in younger patients and characterized by rapid progression, with the majority of patients achieving complete remission after chemotherapy. In contrast, ALK -ALCL generally affects older patients, presenting with similar clinical features but a poorer prognosis. Genetic rearrangements, such as DUSP22 and TP63, have a significant impact on patient survival. The current treatment regimen primarily involves CHOP, but its efficacy is limited, especially in relapsed cases. Consequently, there is an urgent need to improve frontline and salvage treatment options. Future research should focus on the integration of targeted therapies with molecular mechanisms to enhance therapeutic outcomes and survival rates. Understanding the subtypes and molecular characteristics of ALCL is essential for optimizing therapeutic strategies. Therefore, this review summarizes recent research advances on ALK +/- ALCL to provide insights for optimizing treatment approaches. ALK +/-间变性大细胞淋巴瘤的研究进展. 间变性大细胞淋巴瘤(ALCL)是一种罕见的CD30阳性T细胞非霍奇金淋巴瘤,主要分为 ALK基因重排阳性( ALK +)和 ALK基因重排阴性( ALK -)两种亚型。 ALK +ALCL常见于年轻患者,具有快速发展的特征,多数患者在化疗后可得到完全缓解。相比之下, ALK -ALCL患者年龄通常较大,虽然临床表现相似,但预后较差,相关基因重排(如DUSP22 和TP63)对患者生存率有显著影响。目前的治疗方案以CHOP为主,但疗效有限,特别是在复发病例中。因此,亟需改进一线治疗及挽救性方案。未来的研究应关注靶向治疗与分子机制的结合,以提高患者的治疗效果和生存 率。而理解ALCL的亚型和分子特征对于优化治疗策略至关重要,因此,本文对 ALK +/-ALCL的研究进展进行综述,以期为优化治疗策略提供参考。 .

#3

Flaccid Lower Limb Paraparesis Despite Infiltration of the Spinal Cord by a Secondary Central Nervous System T-cell Non-Hodgkin Lymphoma With Syringomyelia: A Case Report.

Cureus2026 Feb

Secondary central nervous system (CNS) lymphomas typically present with weakness, spasticity, exaggerated tendon reflexes, and pyramidal signs. A patient with progressive flaccid paraparesis of the lower extremities despite carcinomatosis and infiltration of T-cell non-Hodgkin lymphoma (NHL) into the spinal cord has not been described to date. A 71-year-old man was diagnosed with T-cell NHL not otherwise specified, stage 4B, and an international prognostic index of 5 based on a lymph node biopsy and bone marrow puncture. The patient received three cycles of cyclophosphamide, hydroxydaunorubicin, vincristine, and prednisone (CHOP) chemotherapy, which had to be discontinued due to thrombocytopenia and the onset of paraparesis of the lower extremities. Examination of the paraparesis revealed lymphoma infiltrates in the brain and spinal cord. Despite these CNS lesions, the patient did not exhibit spasticity, pyramidal signs, or increased reflexes; rather, hypotonia and a general absence of reflexes were observed. These were attributed to axonal motor neuropathy due to vincristine toxicity. T-cell NHL may not be suppressed by CHOP chemotherapy, may spread secondarily to the CNS, and may infiltrate the brain and spinal cord, manifesting as paraparesis, incontinence, muscle hypotonia, and decreased tendon reflexes. Secondary CNS lymphoma does not necessarily have to be accompanied by hyperreflexia, pyramidal signs, and spasticity if the peripheral nerves are severely affected by vincristine toxicity.

#4

Retrospective analysis of clinical characteristics, treatment outcomes and prognosis of patients with natural killer/T-cell lymphoma.

Oncology letters2026 Jan

Natural killer/T-cell lymphoma (NKTCL) is the most common T-cell non-Hodgkin lymphoma in China; however, the clinical characteristics and optimal treatment remain unclear. The present study aimed to retrospectively assess the clinical features of patients with NKTCL and to determine treatment strategies. A total of 200 patients who were diagnosed with NKTCL and admitted to the Cancer Center, Union Hospital (Wuhan, China) between June 2013 and June 2022 were identified. Clinical data from these patients were collected and analyzed to evaluate the clinical features, treatment outcomes and prognostic factors. A total of 171 patients diagnosed with NKTCL were ultimately enrolled. Patients were diagnosed at a median age of 45 years and had a male-to-female ratio of 2:1. Out of the 171 patients, 117 were in the early stage (stage I/II; 68.4%) and 54 were in the advanced stage (stage III/IV; 31.6%) of NKTCL. Among these patients, B symptoms were present in 60.2% of patients (103/171) and the Eastern Cooperative Oncology Group performance status was scored as 0-1 in 93.6% of patients (160/171). Most patients had a low-to-moderate risk of international prognostic index (147/171; 86.0%), prognostic index for NKTCL (PINK; 122/171; 71.3%) and PINK with extended features (112/171; 65.5%). At baseline, it was demonstrated that Epstein-Barr virus DNA was positive in the serum of 166/171 patients (2.9%). Combined chemotherapy and radiotherapy at 54 Gy revealed a promising effect with the highest overall response rate (ORR) of 98.2%, a 4-year progression-free survival (PFS) rate of 82.5% [95% confidence interval (CI), 75.1-89.9%] and an overall survival (OS) rate of 92.2% (95% CI, 86.9-97.5%) for the early-stage group. Pegasparase-based chemotherapy was used to treat advanced-stage patients, resulting in a 4-year PFS rate of 62.3% (95% CI, 47.4-77.2%) and an OS rate of 63.5% (95% CI, 48.2-78.8%). The regimen containing the programmed cell death protein 1 inhibitor camrelizumab demonstrated an ORR of 100% in both the early- and advanced-stage cohorts. Moreover, stage and an age of >60 years were independent prognostic factors for poor PFS, whilst stage and elevated lactate dehydrogenase were independent prognostic factors for poor OS. In conclusion, the results indicated promising efficacy for pegaspargase-based chemotherapy in terms of response rates and survival outcomes for both early- and advanced-stage patients. However, further refinement of treatment strategies is needed.

#5

Advances and updates in pediatric anaplastic large cell lymphoma.

Blood advances2025 Oct 14

Anaplastic large cell lymphoma (ALCL) is a rare form of mature T-cell non-Hodgkin lymphoma. In pediatric patients, most cases are anaplastic lymphoma kinase (ALK) positive. Despite intensive multiagent chemotherapy regimens, treatment failure rates remain at 25% to 30%. Recent advancements in targeted therapies, notably ALK inhibitors and the anti-CD30 antibody-drug conjugate brentuximab vedotin have demonstrated substantial activity in relapsed and refractory settings. Molecular detection of minimal disseminated disease (MDD) and minimal residual disease (MRD) offers improved prognostic stratification. For patients with relapsed or refractory disease, targeted therapies have increased treatment options, but more work needs to be done to define optimal treatment regimens, duration, and need for hematopoietic stem cell transplantation in this group. Immune therapies such as checkpoint inhibitors or chimeric antigen receptor T-cell therapy provide additional therapeutic options. Incorporating targeted therapies and MDD/MRD assessments into clinical trials could significantly improve outcomes for pediatric and adolescent patients with ALCL.

Publicações recentes

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📚 EuropePMC80 artigos no totalmostrando 132

2026

[Research Progress in ALK+/- Anaplastic Large Cell Lymphoma--Review].

Zhongguo shi yan xue ye xue za zhi
2026

Flaccid Lower Limb Paraparesis Despite Infiltration of the Spinal Cord by a Secondary Central Nervous System T-cell Non-Hodgkin Lymphoma With Syringomyelia: A Case Report.

Cureus
2025

Case Report: diffuse entire gastrointestinal tract involvement of ALK-positive anaplastic large cell lymphoma harboring JAK-STAT pathway mutations in an adolescent with leukemoid reaction.

Frontiers in oncology
2025

Anaplastic Lymphoma Kinase (ALK)-Positive Anaplastic Large Cell Lymphoma Presenting as an Axillary Breast Abscess in a Young Woman: A Case Report.

Cureus
2026

Retrospective analysis of clinical characteristics, treatment outcomes and prognosis of patients with natural killer/T-cell lymphoma.

Oncology letters
2025

Incidence trends and survival analysis of enteropathy-associated T-cell lymphoma.

Frontiers in immunology
2026

Incidence of Hypertension During Induction Therapy in Children with Acute Lymphoblastic Leukemia.

Indian pediatrics
2025

Sweet Syndrome-like Dermatosis as a Precursor to Overlapping Hematologic Malignancies: A Case Report and Review.

Journal of clinical medicine
2025

Advances and updates in pediatric anaplastic large cell lymphoma.

Blood advances
2025

BIA-ALCL diagnosis: the relevance of cytology and flow cytometry in periprosthetic fluid analysis.

Annales de biologie clinique
2025

Coexistence of T-Cell Lymphoblastic Lymphoma and Ichthyosis Vulgaris: A Case Report.

Case reports in oncological medicine
2024

Long-term survival and clinical implications of allogeneic stem cell transplantation in relapse/refractory lymphoma: A 20-year Singapore experience.

Annals of the Academy of Medicine, Singapore
2025

HSP-CAR30 with a high proportion of less-differentiated T cells promotes durable responses in refractory CD30+ lymphoma.

Blood
2025

Peripheral T-cell lymphoma-NOS in children and adolescents: a review from the Children's Oncology Group NHL Committee.

Blood advances
2025

Enhancement of High-Dose Chemotherapy and Autologous SCT with the PARP Inhibitor Olaparib for Refractory Lymphoma.

Clinical cancer research : an official journal of the American Association for Cancer Research
2025

Spontaneous regression in mature T-cell non-Hodgkin lymphoma.

Expert review of hematology
2024

Tumour assessment of ROR1 levels in various adult leukaemia and lymphoma types.

PloS one
2024

Breast Implant-Associated Anaplastic Large Cell Lymphoma: Where Hematology and Plastic Surgery Meet.

Clinical lymphoma, myeloma &amp; leukemia
2024

Flow Cytometry Analysis in Breast Implant-Associated Anaplastic Large Cell Lymphoma: Three Case Reports.

International journal of molecular sciences
2024

Disseminated Anaplastic Lymphoma Kinase (ALK)-Positive T-cell Lymphoma Involving the Uterus and Cervix: A Case Report.

Cureus
2024

Case report: The utilization of crizotinib and brentuximab vedotin as a bridge to autologous stem cell transplantation and followed by CD30-directed CAR-T cell therapy in relapsed/refractory ALK+ ALCL.

Frontiers in immunology
2023

Incidental Bystander or Essential Culprit: A Systematic Review of Bacterial Significance in the Pathogenesis of Breast Implant-Associated Anaplastic Large Cell Lymphoma.

International journal of molecular sciences
2023

A pancreatic puzzle: A T-cell non-Hodgkin lymphoma in a 3-year-old child with acute pancreatitis.

Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology
2024

Intraoperative pathologic diagnosis of central nervous system lymphomas: A comparison of frozen and permanent section diagnoses, and the significance of preoperative imaging.

Annals of diagnostic pathology
2023

Update on the Pathogenesis of Enteropathy-Associated T-Cell Lymphoma.

Diagnostics (Basel, Switzerland)
2023

Chimeric Antigen Receptor T Cells in Hodgkin and T-Cell Lymphomas.

Hematology/oncology clinics of North America
2023

A phase 2 study of pembrolizumab after autologous stem cell transplantation in patients with T-cell non-Hodgkin lymphoma.

Blood
2023

Zidovudine and Interferon Alfa based regimens for the treatment of adult T-cell leukemia/lymphoma (ATLL): a systematic review and meta-analysis.

Virology journal
2023

Brentuximab vedotin use in pediatric anaplastic large cell lymphoma.

Frontiers in immunology
2023

Textured vs. Smooth Breast Implants Using the Jones Criteria-What Is the Currently Available Evidence for BIA-ALCL?: A Systematic Review.

Journal of personalized medicine
2023

Massive myocardial infiltration by primary anaplastic T-cell lymphoma: a case report.

European heart journal. Case reports
2023

A Rare Case of Metachronous Peripheral T-cell Non-Hodgkin Lymphoma Following Epstein-barr Virus-positive Diffuse Large B-cell Lymphoma, Not Otherwise Specified.

Oman medical journal
2023

Are we there yet? cellular therapies for cutaneous T cell lymphoma.

Current research in translational medicine
2023

Increasing Risk of Lymphoma Over Time in Crohn's Disease but Not in Ulcerative Colitis: A Scandinavian Cohort Study.

Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association
2023

First-Line Therapy for Nodal T-cell Non-Hodgkin Lymphomas: an Unmet Need in Hematology.

Current oncology reports
2023

Potential miRNA-gene interactions determining progression of various ATLL cancer subtypes after infection by HTLV-1 oncovirus.

BMC medical genomics
2023

Primary Gastrointestinal T-cell Lymphoma Presenting as Spontaneous Perforation Peritonitis.

Cureus
2022

Safety and efficacy of dual PI3K-δ, γ inhibitor, duvelisib in patients with relapsed or refractory lymphoid neoplasms: A systematic review and meta-analysis of prospective clinical trials.

Frontiers in immunology
2023

SBNO2 is a critical mediator of STAT3-driven hematological malignancies.

Blood
2022

Surgical management of monomorphic epitheliotropic intestinal T-cell lymphoma followed by chemotherapy and stem-cell transplant: A case report and review of the literature.

World journal of gastrointestinal oncology
2022

Inter-intra instrument comparison and standardization of a 10-color immunophenotyping for B and T cell non-Hodgkin lymphoma diagnosis and monitoring.

Journal of immunological methods
2022

Hepatosplenic T-Cell Non-Hodgkin Lymphoma Cured with Tandem Autologous and Allogeneic Stem Cell Transplantation.

Chemotherapy
2022

T-Cell Non-Hodgkin Lymphoma of the Ileum Presenting as Perforation and Peritonitis: A Case Report.

Frontiers in surgery
2022

An Updated Approach and Understanding of Breast Implant-Associated Anaplastic Large Cell Lymphoma.

Journal of the National Comprehensive Cancer Network : JNCCN
2022

Sting Is Commonly and Differentially Expressed in T- and Nk-Cell but Not B-Cell Non-Hodgkin Lymphomas.

Cancers
2023

Immunohistochemical evaluation and prognostic value of monocarboxylate transporter 1 (MCT1) and 4 (MCT4) in T-cell non-Hodgkin lymphoma.

Clinical and experimental medicine
2022

Impact of conditioning regimen intensity on the outcomes of peripheral T-cell lymphoma, anaplastic large cell lymphoma and angioimmunoblastic T-cell lymphoma patients undergoing allogeneic transplant.

British journal of haematology
2022

Coexistence of T-Large Granular Lymphocyte Leukemia and Peripheral T Cell Lymphoma-NOS with Indolent Behavior.

Mediterranean journal of hematology and infectious diseases
2022

Severe Generalized Weakness, Paraplegia following administration of Oxaliplatin in a patient with refractory T-cell non-Hodgkin lymphoma: A case report.

Journal of oncology pharmacy practice : official publication of the International Society of Oncology Pharmacy Practitioners
2021

Ukrainian prospective study in patients with T-cell non-Hodgkin lymphomas.

Experimental oncology
2021

Primary gastrointestinal anaplastic large cell lymphoma: A critical reappraisal with a systematic review of the world literature.

Journal of cancer research and therapeutics
2022

Prognostic evaluation models for primary thyroid lymphoma, based on the SEER database and an external validation cohort.

Journal of endocrinological investigation
2021

Metagenomic analysis to identify novel infectious agents in systemic anaplastic large cell lymphoma.

Infectious agents and cancer
2022

BIA-ALCL diagnosis on CytoLyt fixed ThinPrep, cell block and immunohistochemistry.

Diagnostic cytopathology
2022

Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL): a good practice guide, pictorial review, and new perspectives.

Clinical radiology
2022

Flow Cytometric Detection of the Double-Positive (CD4+CD8+)/PD-1bright T-Cell Subset Is Useful in Diagnosing Nodular Lymphocyte-Predominant Hodgkin Lymphoma.

Archives of pathology &amp; laboratory medicine
2021

The management of breast implant-associated anaplastic large cell lymphoma in the setting of pregnancy: seeking for clinical practice guidelines.

Archives of plastic surgery
2021

Epstein-Barr virus positive diffuse large B-cell lymphoma transformed into angioimmunoblastic T-cell lymphoma after treatment.

Clinical case reports
2021

MRI features of breast implant-associated anaplastic large cell lymphoma.

The British journal of radiology
2021

Post-Chemotherapy Rebound Thymic Hyperplasia Mimicking Relapse in Breast Implant-Associated Anaplastic Large Cell Lymphoma: A Case Report.

Oncology research and treatment
2021

How I Diagnose Anaplastic Large Cell Lymphoma.

American journal of clinical pathology
2022

Two young patients with extranodal natural killer/T-cell non-Hodgkin lymphoma, nasal-type (ENKTL-NT) masquerading inflammatory processes: A case series.

Taiwan journal of ophthalmology
2021

A randomized phase 3 trial of autologous vs allogeneic transplantation as part of first-line therapy in poor-risk peripheral T-NHL.

Blood
2021

UK Guidelines on the Diagnosis and Treatment of Breast Implant-Associated Anaplastic Large Cell Lymphoma (BIA-ALCL) on behalf of the Medicines and Healthcare products Regulatory Agency (MHRA) Plastic, Reconstructive and Aesthetic Surgery Expert Advisory Group (PRASEAG).

Journal of plastic, reconstructive &amp; aesthetic surgery : JPRAS
2021

Breast implant-associated anaplastic large-cell lymphoma (BIA-ALCL).

Yeungnam University journal of medicine
2021

NK/T-cell non-Hodgkin lymphoma: Case report and review of the literature.

Experimental and therapeutic medicine
2021

UK Guidelines on the Diagnosis and Treatment of Breast Implant-Associated Anaplastic Large Cell Lymphoma on behalf of the Medicines and Healthcare products Regulatory Agency Plastic, Reconstructive and Aesthetic Surgery Expert Advisory Group.

British journal of haematology
2021

The effect of PD-1 expression on tumor-associated macrophage in T cell lymphoma.

Clinical &amp; translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico
2021

FANCA, TP53, and del(5q)/RPS14 alterations in a patient with T-cell non-Hodgkin lymphoma and concomitant Fanconi anemia and Li-Fraumeni syndrome.

Cancer genetics
2020

A case of metachronous peripheral T-Cell non-Hodgkin lymphoma following chemotherapy for Hodgkin disease successfully treated with brentuximab vedotin.

Clinical case reports
2020

Hematopoietic Cell Transplantation and Adoptive Cell Therapy in Peripheral T Cell Lymphoma.

Current hematologic malignancy reports
2020

Improved outcomes with allogeneic compared with autologous stem cell transplantation in aggressive T-cell lymphoma.

European journal of haematology
2020

Predicting worse survival for newly diagnosed T cell lymphoma based on the decreased baseline CD16-/CD16 + monocyte ratio.

Scientific reports
2019

Gamma-Delta T-Cell Acute Lymphoblastic Leukemia/Lymphoma: Immunophenotype of Three Adult Cases.

Journal of hematology
2020

Aberrant CD3-Positive, CD8-Low, CD7-Negative Lymphocytes May Appear During Viral Infections and Mimic Peripheral T-Cell Lymphoma.

Diagnostics (Basel, Switzerland)
2020

Primary dural lymphomas: Clinical presentation, management, and outcome.

Cancer
2020

Diagnostic utility of STAT6YE361 expression in classical Hodgkin lymphoma and related entities.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2019

Refined Disease Risk Index for Hematological Malignancies, Including Rare Disorders, After Allogeneic Stem Cell Transplantation.

Transplantation proceedings
2019

A Diagnostic Dilemma of Sinonasal T Cell Lymphoma: Report of A Unique Case and Literature Review.

The Gulf journal of oncology
2019

The application of antigen receptor gene rearrangement of BIOMED-2 in the pathologic diagnosis of 348 cases with non-Hodgkin lymphoma in a single institution in Southwest of China.

Pathology, research and practice
2019

Identification of a new family of pyrazolo[3,4-d]pyrimidine derivatives as multitarget Fyn-Blk-Lyn inhibitors active on B- and T-lymphoma cell lines.

European journal of medicinal chemistry
2019

Beneficial effect of consolidative radiotherapy for patients with lymphoma and skeletal involvement.

Medicine
2019

Cytological features of breast implant-associated anaplastic large cell lymphoma in pleural effusion.

Diagnostic cytopathology
2019

Peripheral T-Cell Lymphoma: Moving Toward Targeted Therapies.

Hematology/oncology clinics of North America
2019

Ontogeny, Genetics, Molecular Biology, and Classification of B- and T-Cell Non-Hodgkin Lymphoma.

Hematology/oncology clinics of North America
2019

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) associated with or without lymphoma: Comparison of clinical features and risk factors suggestive of underlying lymphomas.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2019

The prognostic value of toll-like receptor5 and programmed cell death-ligand1 in patients with peripheral T-cell non-Hodgkin lymphoma.

Leukemia &amp; lymphoma
2020

Characteristics, treatment patterns, prognostic determinants and outcome of peripheral T cell lymphoma and natural killer/T cell non-Hodgkin Lymphoma in older patients: The result of the nationwide multi-institutional registry Thai Lymphoma Study Group.

Journal of geriatric oncology
2019

Genetics of Breast Implant-Associated Anaplastic Large Cell Lymphoma (BIA-ALCL).

Aesthetic surgery journal
2019

From bench to bedside: the past, present and future of therapy for systemic paediatric ALCL, ALK.

British journal of haematology
2019

Autologous transplantation as consolidation for high risk aggressive T-cell non-Hodgkin lymphoma: a SWOG 9704 intergroup trial subgroup analysis.

Leukemia &amp; lymphoma
2019

A phase II study of a modified hyper-CVAD frontline therapy for patients with adverse risk diffuse large B-cell and peripheral T-cell non-Hodgkin lymphoma.

Leukemia &amp; lymphoma
2018

Alteration of gene expression profile in CD3+ T-cells after downregulating MALT1.

ImmunoTargets and therapy
2018

Peripheral T-cell non-Hodgkin lymphoma manifesting as a primary lesion on the lip: A rare case report.

Special care in dentistry : official publication of the American Association of Hospital Dentists, the Academy of Dentistry for the Handicapped, and the American Society for Geriatric Dentistry
2018

Current Immunotherapeutic Approaches in T Cell Non-Hodgkin Lymphomas.

Cancers
2018

How I treat breast implant-associated anaplastic large cell lymphoma.

Blood
2018

Expression of PD-1 on peripheral blood Treg cells is related to the diagnosis, prognosis and treatment of T cell non-Hodgkin lymphoma.

Leukemia research
2018

Aggressive Angioimmunoblastic T Cell Lymphomas (AITL) with Soft Tissue Extranodal Mass Varied Histopathological Patterns with Peripheral Blood, Bone Marrow, and Splenic Involvement and Review of Literature.

Indian journal of surgical oncology
2018

Evaluation of tenascin-C by tenatumomab in T-cell non-Hodgkin lymphomas identifies a new target for radioimmunotherapy.

Oncotarget
2018

Umbralisib, a novel PI3Kδ and casein kinase-1ε inhibitor, in relapsed or refractory chronic lymphocytic leukaemia and lymphoma: an open-label, phase 1, dose-escalation, first-in-human study.

The Lancet. Oncology
2018

Eosinophilic infiltrate resembling eosinophilic cellulitis (Wells syndrome) in a patient with mycosis fungoides.

Dermatology online journal
2018

Breast necrosis in a patient with primary T-cell non-Hodgkin lymphoma.

Cirugia espanola
2018

A Phase I Dose-Escalation Study of Clofarabine in Patients with Relapsed or Refractory Low-Grade or Intermediate-Grade B-Cell or T-Cell Lymphoma.

The oncologist
2018

Clinicopathological characteristics of T-cell non-Hodgkin lymphoma arising in patients with immunodeficiencies: a single-center case series of 25 patients and a review of the literature.

Haematologica
2017

Blueprint unknown: a case for multidisciplinary management of advanced penile mycosis fungoides.

The Canadian journal of urology
2017

Co-targeting aurora kinase with PD-L1 and PI3K abrogates immune checkpoint mediated proliferation in peripheral T-cell lymphoma: a novel therapeutic strategy.

Oncotarget
2017

Occupational ultraviolet exposure and risk of non-Hodgkin's lymphomas: a meta-analysis.

Oncotarget
2018

Autoimmune manifestations associated with lymphoma: characteristics and outcome in a multicenter retrospective cohort study.

Leukemia &amp; lymphoma
2017

Myxoid variant of primary cutaneous anaplastic large cell lymphoma: First 2 cases.

Journal of cutaneous pathology
2018

Cardiac Hematological Malignancies: Typical Growth Patterns, Imaging Features, and Clinical Outcome.

Angiology
2017

Patients with Fever of Unknown Origin and Splenomegaly: Diagnostic Value of Splenectomy and Preoperative Risk Factors Suggestive of Underlying Lymphomas.

Acta haematologica
2017

Demodicosis in Pediatric Cancer.

Journal of pediatric hematology/oncology
2017

The prognostic value of 18-FDG positron emission tomography in T cell non-Hodgkin lymphoma.

Blood cancer journal
2017

An audit of patients with mature T-cell non-Hodgkin lymphoma by transplant status in Tasmania.

Internal medicine journal
2017

Accuracy of 18-F FDG PET/CT to detect bone marrow clearance in patients with peripheral T-cell lymphoma - tissue remains the issue.

Leukemia &amp; lymphoma
2016

The Activating NKG2C Receptor Is Significantly Reduced in NK Cells after Allogeneic Stem Cell Transplantation in Patients with Severe Graft-versus-Host Disease.

International journal of molecular sciences
2016

Immune Thrombocytopenia in a Child with T Cell Lymphoblastic Lymphoma.

Case reports in hematology
2016

An unusual extranodal T-cell non-Hodgkin lymphoma.

Lancet (London, England)
2016

[Intestinal infiltration of high-grade large T-cell non-Hodgkin lymphoma with cyclin-D1 overexpression and aberrant CD79a expression in a patient with a diagnosis of tumour stage mycosis fungoides].

Gastroenterologia y hepatologia
2016

Adult systemic anaplastic large-cell lymphoma: recommendations for diagnosis and management.

Expert review of hematology
2015

Dose-Effect Relationship of Alkylating Agents on Testicular Function in Male Survivors of Childhood Lymphoma.

Pediatric hematology and oncology
2016

Identification of a new subclass of ALK-negative ALCL expressing aberrant levels of ERBB4 transcripts.

Blood
2015

[Constitutional mismatch repair deficiency syndrome].

Nederlands tijdschrift voor geneeskunde
2015

T-cell lymphoma with von Hippel-Lindau disease: a rare case report and review of literature.

International journal of clinical and experimental pathology
2015

[T-cell non-Hodgkin lymphoma as a rare mycosis fungoide complication].

Revista espanola de geriatria y gerontologia
2016

Genetics of anaplastic large cell lymphoma.

Leukemia &amp; lymphoma
2015

Primary T-Cell Non-Hodgkin Lymphoma of the Vagina.

Case reports in obstetrics and gynecology
2015

Phase II Intergroup Trial of Alisertib in Relapsed and Refractory Peripheral T-Cell Lymphoma and Transformed Mycosis Fungoides: SWOG 1108.

Journal of clinical oncology : official journal of the American Society of Clinical Oncology
2015

Constitutive control of AKT1 gene expression by JUNB/CJUN in ALK+ anaplastic large-cell lymphoma: a novel crosstalk mechanism.

Leukemia
2015

Enteropathy-associated T-cell lymphoma: improving treatment strategies.

Digestive diseases (Basel, Switzerland)
2015

[Safety of polyethylene glycol conjugated L-asparaginase in patients with acute lymphoblastic leukemia and T cell non-Hodgkin lymphoma].

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi
2015

Treatment response in enteropathy associated T-cell lymphoma; survival in a large multicenter cohort.

American journal of hematology

Associações

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Comunidades

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Ainda não existe comunidade no Raras para Linfoma não-Hodgkin de células T

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Incidence of Hypertension During Induction Therapy in Children with Acute Lymphoblastic Leukemia.
    Indian pediatrics· 2026· PMID 41060554mais citado
  2. [Research Progress in ALK+/- Anaplastic Large Cell Lymphoma--Review].
    Zhongguo shi yan xue ye xue za zhi· 2026· PMID 41846374mais citado
  3. Flaccid Lower Limb Paraparesis Despite Infiltration of the Spinal Cord by a Secondary Central Nervous System T-cell Non-Hodgkin Lymphoma With Syringomyelia: A Case Report.
    Cureus· 2026· PMID 41804404mais citado
  4. Retrospective analysis of clinical characteristics, treatment outcomes and prognosis of patients with natural killer/T-cell lymphoma.
    Oncology letters· 2026· PMID 41277910mais citado
  5. Advances and updates in pediatric anaplastic large cell lymphoma.
    Blood advances· 2025· PMID 40690755mais citado
  6. Case Report: A case of jejunal T-cell non-Hodgkin lymphoma with secondary bone involvement presenting as gastrointestinal perforation.
    Front Med (Lausanne)· 2026· PMID 41958549recente
  7. Case Report: diffuse entire gastrointestinal tract involvement of ALK-positive anaplastic large cell lymphoma harboring JAK-STAT pathway mutations in an adolescent with leukemoid reaction.
    Front Oncol· 2025· PMID 41584605recente
  8. Anaplastic Lymphoma Kinase (ALK)-Positive Anaplastic Large Cell Lymphoma Presenting as an Axillary Breast Abscess in a Young Woman: A Case Report.
    Cureus· 2025· PMID 41445989recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:171918(Orphanet)
  2. MONDO:0015760(MONDO)
  3. GARD:20133(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Q7667896(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Linfoma não-Hodgkin de células T
Compêndio · Raras BR

Linfoma não-Hodgkin de células T

ORPHA:171918 · MONDO:0015760
Prevalência
1-9 / 1 000 000
Ensaios
38 ativos
Medicamentos
10 registrados
Prevalência
0.99 (Europe)
MedGen
UMLS
C0079772
EuropePMC
Wikidata
Papers 10a
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