Pular para o conteúdo
Linfoma B difuso de grandes células positivo para o vírus Epstein-Barr
ORPHA:289661CID-10 · C83.3CID-11 · 2A81.6DOENÇA RARA
Esta doença tem causa genética. Recomendamos procurar um médico geneticista para diagnóstico, exames genéticos e aconselhamento familiar.
Início adultaHerança Unknown
Também conhecida comoDLBCLEBVNOS
Sinônimos clínicos: Linfoma B difuso de grandes células positivo para o vírus Epstein-Barr sem outra especificação · DLBCL EBV-positivo · Linfoma B difuso de grandes células positivo para o vírus Epstein-Barr, NOS

Uma forma rara de linfoma difuso de grandes células B que ocorre mais comumente em pacientes com mais de 50 anos de idade (geralmente entre 70 e 75 anos de idade), sem imunodeficiência evidente, e que se apresenta com envolvimento nodal e extranodal (em locais como estômago, pulmão, pele e pâncreas) e sintomas B (febre, suores noturnos, perda de peso). O tumor é caracterizado por um curso agressivo e uma taxa de sobrevida curta.

Em construçãohistóricoSugerir correção
Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

Mantido pelo Disease Twin100% com fonte · atualizado 27/09/2026Revisão médica pendente
Informacoes curadas por IA — podem conter imprecisoes

Visão geral

O Linfoma B difuso de grandes células positivo para o vírus Epstein-Barr é uma forma rara de linfoma difuso de grandes células B associada ao código CID-10 C83.3. Trata-se de uma condição com prevalência desconhecida, cujo início dos sintomas ocorre tipicamente em adultos e idosos, sendo mais frequente em indivíduos com mais de 50 anos de idade (geralmente entre 70 e 75 anos), sem imunodeficiência evidente.[1]

Sinais e sintomas

O quadro clínico apresenta-se com envolvimento tanto nodal quanto extranodal, atingindo locais como estômago, pulmão, pele e pâncreas. Os pacientes frequentemente manifestam sintomas constitucionais B, caracterizados por febre, suores noturnos e perda de peso.[1]

Causas genéticas

O padrão de herança genética é classificado como não aplicável para esta doença e não há genes causais específicos listados nos registros.[1]

Diagnóstico

Existe registro de 1 teste genético disponível associado à condição nos dados cadastrados.[1]

Tratamento e manejo

No âmbito do Sistema Único de Saúde (SUS), o Protocolo Clínico e Diretrizes Terapêuticas (PCDT) vigente para Linfoma Difuso de Grandes Células B (Portaria SAS/MS nº 956, de 26 de setembro de 2014) prevê a dispensação conforme a autonomia do serviço de saúde.[2]

Prognóstico e qualidade de vida

O tumor é caracterizado por um curso biológico agressivo e está associado a uma taxa de sobrevida curta.[1]

Fontes numeradas em Referências.

Conteúdo informativo gerado e mantido automaticamente a partir de fontes oficiais (Orphanet, HPO, OMIM, SUS). Não substitui avaliação médica.

💬
EM LINGUAGEM SIMPLES

O linfoma B difuso de grandes células positivo para o vírus Epstein-Barr é um tipo raro e agressivo de câncer que surge nas células de defesa do organismo. A condição afeta com maior frequência adultos e idosos acima de 50 anos, causando febre, suores noturnos, perda de peso e aumento de gânglios ou alterações em órgãos como estômago e pulmões. No SUS, o tratamento é oferecido conforme a autonomia de cada serviço de oncologia especializado, sob as diretrizes do PCDT de Linfoma Difuso de Grandes Células B. Por ter uma progressão rápida, o diagnóstico ágil e o acompanhamento próximo em centros hospitalares são fundamentais para o paciente.

Informacoes curadas por IA — podem conter imprecisoes
[1] Orphanet [17] PCDT vigente "Linfoma Difuso de Grande…
📋
Informacoes curadas por IA — podem conter imprecisoes

Uma forma rara de linfoma difuso de grandes células B que ocorre mais comumente em pacientes com mais de 50 anos de idade (geralmente entre 70 e 75 anos de idade), sem imunodeficiência evidente, e que se apresenta com envolvimento nodal e extranodal (em locais como estômago, pulmão, pele e pâncreas) e sintomas B (febre, suores noturnos, perda de peso). O tumor é caracterizado por um curso agressivo e uma taxa de sobrevida curta.

Pesquisas ativas
2 ensaios
9 total registrados no ClinicalTrials.gov
Publicações científicas
160 artigos
Último publicado: 2026 Mar 3

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Adult
+ elderly
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: C83.3
Você se identifica com essa condição?
O Raras está aqui pra te apoiar — com ou sem diagnóstico

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

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

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico160PubMed
Últimos 10 anos117publicações
Pico202016 papers
Linha do tempo
2026Hoje · 2026🧪 2016Primeiro ensaio clínico📈 2020Ano 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

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

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.
2Fase 29
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 9 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Linfoma B difuso de grandes células positivo para o vírus Epstein-Barr

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Onde estão os ensaios

Com ensaio aberto em 2 países. O ponto verde marca onde há vaga agora.

🟢 Recrutando agora

1 pesquisa recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

9 ensaios clínicos encontrados, 2 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
🧪 Está conduzindo uma pesquisa?
Divulgue para pacientes e familiares que acompanham esta doença.
Divulgar pesquisa →

Publicações mais relevantes

Timeline de publicações
118 papers (10 anos)
#1

Long noncoding RNA ADEI/miR-93-3p/STAT3 axis promotes Epstein-Barr virus-positive diffuse large B-cell lymphoma progression and immune evasion through regulating the PD-1/PD-L1 checkpoint.

Cell death &amp; disease2026 Mar 03

Epstein-Barr virus (EBV) is an important pathogenic factor of lymphoma; EBV+diffuse large B-cell lymphoma (DLBCL) has a worse prognosis with standard chemotherapy than EBV-DLBCL. Long noncoding (Lnc)-RNAs are key regulators of cancer pathways and biomarkers of disease. As natural protective carriers of noncoding RNAs, exosomes can stably transmit signals during tumor development. We explored the role of exosomal lncRNAs in the occurrence and development of EBV-related DLBCL. In this study, we identified a novel lncRNA lncADEI, which was upregulated in EBV + DLBCL and was positively correlated with DLBCL cell proliferation and clonogenesis. LncADEI positively regulated STAT3 via miR-93-3P, and STAT3 transcriptionally activated programmed death ligand-1 to promote immune evasion of DLBCL cells. LncADEI could be transferred by exosomes and promote the proliferation and immune evasion of DLBCL. LncADEI was highly expressed in serum exosomes from EBV + DLBCL patients and was associated with worse clinicopathological features. In conclusion, lncADEI participated in the progression and immune evasion of EBV + DLBCL and was differentially expressed in serumal exosomes. LncADEI may be a promising strategy for treating EBV-associated lymphoid malignancies.

#2

ORY1001 enhances anti-tumor immunity via CXCL10-mediated macrophage modulation in EBV-positive DLBCL.

Cell communication and signaling : CCS2026 Jan 15

Epstein-Barr virus-positive diffuse large B-cell lymphoma (EBV+ DLBCL) is an aggressive malignancy characterized by an immunosuppressive tumor microenvironment (TME), where tumor-associated macrophages (TAMs) often exhibit an M2-like phenotype that facilitates immune evasion. Here, we show that the LSD1 inhibitor ORY1001 reprograms the TME of EBV+ DLBCL through CXCL10-mediated modulation of macrophage polarization. Using a reductionist model in which EBV-negative DLBCL cell lines were transfected with LMP1, we demonstrate that ORY1001 induces robust expression of CXCL10, TNF-α and IL-6 in LMP1+ Riva cells, promoting M1 macrophage differentiation and reprogramming M2 macrophages toward a pro-inflammatory phenotype. This CXCL10-driven macrophage reprogramming was also observed in an additional LMP1+ DLBCL line (U2932) and in primary EBV+ DLBCL cells, in which ORY1001 selectively increased CXCL10 levels in tumor-cell supernatants, and conditioned media from these cultures—but not direct ORY1001 exposure—drove M1 polarization of THP-1 and primary CD14+ monocytes. These effects were dependent on CXCL10-CXCR3 signaling, as pharmacological inhibition of CXCR3 abrogated M1 polarization. In vivo, ORY1001 suppressed tumor growth in LMP1-expressing A20 lymphoma models, increased CXCL10 levels, and enhanced M1 macrophage infiltration. Furthermore, ORY1001 synergized with anti–PD-1 therapy to enhance T-cell activation, granzyme B expression, and tumor regression. These findings highlight LSD1 inhibition as a promising immunoepigenetic strategy to remodel the TME and improve the efficacy of immune checkpoint blockade in EBV-associated lymphomas. The online version contains supplementary material available at 10.1186/s12964-025-02614-0.

#3

Case Report: Rare pulmonary infection and cytomegalovirus retinitis revealed a case of lymphoma.

Frontiers in medicine2026

Recurrent cytomegalovirus retinitis (CMVR) and rare opportunistic pulmonary infections may be the initial manifestations of underlying immunodeficiency caused by occult hematologic malignancy. Epstein-Barr virus-positive diffuse large B-cell lymphoma, not otherwise specified (EBV+DLBCL-NOS) is an aggressive lymphoma associated with immune dysfunction, predisposing patients to severe opportunistic infections, including CMVR. However, pulmonary co-infection with Tropheryma whipplei and Penicillium digitatum has not been previously described as a presenting feature of EBV+ DLBCL-NOS. A 66-year-old male presented with blurred vision and was diagnosed with CMVR, with profoundly low CD4+ T-cell counts (102 cells/μL) and high cytomegalovirus (CMV) DNA levels in blood and aqueous humor. He initially responded to ganciclovir, but CMVR recurred five months later, accompanied by new pulmonary nodules. Despite negative conventional microbiological tests, metagenomic next-generation sequencing (mNGS) of bronchoalveolar lavage fluid identified co-infection with Tropheryma whipplei and P. digitatum. Broad-spectrum antimicrobial therapy led to partial clinical improvement, but pulmonary lesions persisted. PET-CT revealed hypermetabolic lung and lymph node lesions, and subsequent lung biopsy confirmed EBV+ DLBCL-NOS. The patient's progressive immunodeficiency, recurrent CMVR, and refractory pulmonary infection were ultimately attributed to underlying lymphoma. This case highlights that severe, unexplained immunodeficiency with recurrent CMVR and rare opportunistic pulmonary infections should prompt a high index of suspicion for underlying hematologic malignancy. mNGS and PET-CT are critical tools in the diagnostic workup, but definitive diagnosis relies on histopathological confirmation. Early recognition of such presentations can prevent delays in diagnosing aggressive lymphomas.

#4

Low hemoglobin as a novel prognostic biomarker in EBV-positive diffuse large B-cell lymphoma patients: a clinical feature analysis of 99 cases from Zhejiang, Southeast China.

Annals of hematology2026 Feb 28

Epstein-Barr virus-positive diffuse large B-cell lymphoma (EBV+DLBCL) is a rare and clinically aggressive subtype of DLBCL, distinguished by its notably unfavorable prognosis. The clinical characteristics of this entity remain insufficiently characterized in the Chinese population. This study retrospectively analyzed 99 cases of EBV+DLBCL with available clinical data from Zhejiang Province, southeastern China. We comprehensively characterized the clinicopathological features of these patients and performed both univariate and multivariate survival analyses to identify independent prognostic factors. Specifically, 76.7% of patients presented with an Eastern Cooperative Oncology Group performance status (ECOG PS) ≥ 2, 75.8% demonstrated extranodal disease involvement, and 47.5% exhibited B symptoms. Immunophenotypic analysis utilizing the Hans algorithm classified 82.8% (82/99) of cases as non-germinal center B-cell-like (non-GCB) cell-of-origin (COO). The overall survival (OS) rate was 45.4%, while the progression-free survival (PFS) rate was 30.3%. The majority of patients (71.7%, 71/99) received R-CHOP-type immunochemotherapy regimens. Both univariate and multivariate analyses identified R-CHOP-type therapy as a significant independent protective factor for both OS (P < 0.001) and PFS (P = 0.001). Multivariate analysis further revealed that hemoglobin (Hb) levels < 90 g/L, indicative of anemia, represented a potential strong adverse prognostic factor for OS, with moderate to severe anemia emerging as a clinically relevant predictor of poorer treatment outcomes. These findings underscore that EBV+DLBCL constitutes a clinically distinct lymphoma subtype associated with suboptimal outcomes under current chemoimmunotherapy. Notably, readily monitored Hb levels may serve as a practical prognostic biomarker for risk stratification in this patient population. The online version contains supplementary material available at 10.1007/s00277-026-06912-6.

#5

Epstein-Barr Virus-positive Diffuse Large B-cell Lymphoma Presenting as Pyrexia of Unknown Origin Complicated by Paraneoplastic Neurological Syndrome.

Annals of African medicine2026 Feb 20

Chikungunya virus (CHIKV) infection is an arboviral disease transmitted by Aedes mosquitoes, typically presenting with fever, rash, and polyarthralgia. Neurological complications such as meningitis are rare and more often seen in neonates and immunocompromised individuals. Hepatic involvement is also uncommon, with only a few reports describing significant hepatitis in the absence of other risk factors. We report the case of a 68-year-old male, previously healthy with no comorbidities, who presented with acute febrile illness followed by severe headache, neck stiffness, and altered sensorium. Laboratory investigations revealed elevated transaminases, and cerebrospinal fluid analysis was consistent with viral meningitis. Serology confirmed CHIKV infection (immunoglobulin M enzyme-linked immunosorbent assay positive). Neuroimaging was unremarkable. The patient was managed with supportive care, intravenous fluids, and close neurological monitoring. Liver function normalized gradually over 2 weeks, and neurological recovery was complete by discharge. This case highlights the rare dual presentation of Chikungunya meningitis and hepatitis in an elderly patient without underlying comorbidities, underscoring the need for clinicians in endemic areas to consider CHIKV in the differential diagnosis of viral meningitis with hepatic dysfunction. RésuméLe virus Chikungunya (CHIKV) est un arbovirus transmis par les moustiques Aedes, se manifestant généralement par une fièvre, une éruption cutanée et des polyarthralgies. Les complications neurologiques, telles que la méningite, demeurent rares et surviennent plus fréquemment chez les nouveau-nés et les personnes immunodéprimées. L’atteinte hépatique est également inhabituelle, avec seulement quelques cas décrits de véritable hépatite en l’absence d’autres facteurs de risque. Nous rapportons le cas d’un homme de 68 ans, auparavant en bonne santé et sans comorbidités, présentant un syndrome fébrile aigu suivi de céphalées intenses, raideur de la nuque et altération de la conscience. Les examens biologiques ont montré une élévation des transaminases, et l’analyse du liquide céphalorachidien indiquait une méningite virale. La sérologie a confirmé l’infection par le CHIKV (test ELISA IgM positif). L’imagerie cérébrale était normale. Le patient a été pris en charge de manière symptomatique avec hydratation intraveineuse et surveillance neurologique rapprochée. La fonction hépatique s’est normalisée progressivement en deux semaines, et la récupération neurologique a été complète au moment de la sortie. Ce cas illustre la présentation rare d’une méningite associée à une hépatite due au Chikungunya chez un patient âgé sans comorbidités, soulignant l’importance, dans les zones endémiques, d’évoquer le CHIKV devant un tableau de méningite virale accompagnée d’une dysfonction hépatique.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC125 artigos no totalmostrando 115

2026

Long noncoding RNA ADEI/miR-93-3p/STAT3 axis promotes Epstein-Barr virus-positive diffuse large B-cell lymphoma progression and immune evasion through regulating the PD-1/PD-L1 checkpoint.

Cell death &amp; disease
2026

Case Report: Rare pulmonary infection and cytomegalovirus retinitis revealed a case of lymphoma.

Frontiers in medicine
2026

Low hemoglobin as a novel prognostic biomarker in EBV-positive diffuse large B-cell lymphoma patients: a clinical feature analysis of 99 cases from Zhejiang, Southeast China.

Annals of hematology
2026

Epstein-Barr Virus-positive Diffuse Large B-cell Lymphoma Presenting as Pyrexia of Unknown Origin Complicated by Paraneoplastic Neurological Syndrome.

Annals of African medicine
2026

Characteristics and prognosis of Epstein Barr virus-positive diffuse large B-cell lymphoma: a retrospective, single-center study.

Hematology (Amsterdam, Netherlands)
2026

Breast Implant-Associated Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma.

Case reports in hematology
2025

Unmasking Epstein-Barr Virus (EBV)-Positive Large B-Cell Lymphoma as an Underlying Trigger in a Post-COVID Hemophagocytic Lymphohistiocytosis Case.

Cureus
2026

Bilateral Breast Implant-Associated Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma: A Case Report and Literature Review.

Annals of plastic surgery
2026

ORY1001 enhances anti-tumor immunity via CXCL10-mediated macrophage modulation in EBV-positive DLBCL.

Cell communication and signaling : CCS
2025

Ulcerative Necrobiosis Lipoidica in the Setting of Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma and Glucose-6-Phosphate Dehydrogenase Deficiency.

Cureus
2025

Successful Use of Haploidentical HSCT in a Child With Schimke Immuno-Osseous Dysplasia Who Developed PTLD After Kidney Transplantation.

Pediatric transplantation
2025

Clinical characteristics and outcomes in Epstein-Barr virus-positive diffuse large B-cell lymphoma: a multicenter retrospective study.

Turkish journal of medical sciences
2025

[Late-onset Epstein-Barr virus-positive diffuse large B-cell lymphoma at the same site after treatment of extranodal NK/T-cell lymphoma].

[Rinsho ketsueki] The Japanese journal of clinical hematology
2025

[Chinese expert consensus on the diagnosis and management of Epstein-Barr virus positive diffuse large B cell lymphoma (2025)].

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi
2025

Peripheral EBV antigen-specific T cell is dysfunctional in Epstein-Barr virus positive diffuse large B-cell lymphoma.

BMC cancer
2025

Spontaneous remission of Epstein-Barr virus positive diffuse large B-cell lymphoma after relapse post axicabtagene ciloleucel chimeric antigen receptor T-cell therapy.

Leukemia &amp; lymphoma
2025

Recurrent Composite Lymphoma in an Elderly Man: A Case of Marginal Zone Lymphoma With Classic Hodgkin Features Treated With Brentuximab and Immunotherapy.

Cureus
2025

Tumor-Associated Sympathetic Nerves Promote the Progression of Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma.

Advanced science (Weinheim, Baden-Wurttemberg, Germany)
2025

Tiger-man Appearance in a Case of DLBCL.

Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, India
2025

EBV-positive diffuse large B cell lymphoma secondary to activated phosphoinositide 3 kinase δ syndrome type 1 (APDS1): a case report and literature review.

Frontiers in immunology
2025

Spatial transcriptomics reveals tumor microenvironment heterogeneity in EBV positive diffuse large B cell lymphoma.

Scientific reports
2024

Epstein-Barr Virus-Positive Diffuse Large B-cell Lymphoma Arising in Ulcerative Colitis: An Instructive Case Report and Literature Review.

Cureus
2024

A Rare Case of Systemic Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma With Hemophagocytic Lymphohistiocytosis in an Immunocompetent Young Man: Potential Diagnostic Pitfall and Therapeutic Challenge.

Journal of medical cases
2024

Hemophagocytic lymphohistiocytosis in a patient with Epstein-Barr virus-positive diffuse large B-cell lymphoma treated with chimeric antigen receptor T-cell therapy.

Immunotherapy
2024

Alemtuzumab monotherapy for T-cell prolymphocytic leukemia: an observational study in Japan.

Journal of clinical and experimental hematopathology : JCEH
2024

A Newly Diagnosed Patient With Human Immunodeficiency Virus (HIV)-Associated Epstein-Barr Virus (EBV)-Positive Diffuse Large B-cell Lymphoma and Esophageal Candidiasis: A Case Report.

Cureus
2024

Spontaneous Regression of Epstein-Barr Virus-Positive Diffuse Large B-cell Lymphoma in an HIV-Positive Patient: A Case Report and Literature Review.

Cureus
2024

Lymphoproliferative disorder progressing after partial remission following immunosuppressive drugs withdrawal in a patient with rheumatoid arthritis.

Modern rheumatology case reports
2024

The biology and treatment of Epstein-Barr virus-positive diffuse large B cell lymphoma, NOS.

Heliyon
2023

Epstein-Barr Virus Positive Diffuse Large B-Cell Lymphoma with Epidermotropism.

Annals of dermatology
2023

Complete remission of aggressive Epstein-Barr virus-positive diffuse large B-cell lymphoma following withdrawal of tacrolimus and low-dose anticancer drugs.

EJHaem
2023

Cutaneous Methotrexate-Related Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma in a Patient with Granulomatous Cutaneous T-Cell Lymphoma: A Case Report and Literature Review.

Clinical, cosmetic and investigational dermatology
2023

[Epstein-Barr virus positive diffuse large B-cell lymphoma complicated with plasma cell myeloma: report of a case].

Zhonghua bing li xue za zhi = Chinese journal of pathology
2023

A rare case of Epstein-Barr virus-positive diffuse large B-cell lymphoma, not otherwise specified, in a patient with ulcerative colitis.

Clinical journal of gastroenterology
2023

EBV-Positive Diffuse Large B-Cell Lymphoma in Association with Polyurethane Textured Breast Implants: Case Report and Literature Overview.

Aesthetic plastic surgery
2023

Necrotic lymphadenopathy in Epstein-Barr virus-positive diffuse large B-cell lymphoma.

QJM : monthly journal of the Association of Physicians
2023

Primary hepatic Epstein-Barr virus-positive diffuse large B-cell lymphoma associated with azathioprine immunosuppression: a case report.

Journal of medical 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

TET2 and LILRB1 mutations are frequent in Epstein-Barr virus-positive diffuse large B-cell lymphoma especially in elderly patients.

Cancer
2023

Epstein-Barr virus-positive diffuse large B-cell lymphoma onset as familial hemophagocytic lymphohistiocytosis in an infant.

Pediatric blood &amp; cancer
2023

Pathology review identifies frequent misdiagnoses in recurrent classic Hodgkin lymphoma in a nationwide cohort: implications for clinical and epidemiological studies.

Haematologica
2022

Angioimmunoblastic T-Cell Lymphoma: A Case Report.

Cureus
2021

CircEAF2 counteracts Epstein-Barr virus-positive diffuse large B-cell lymphoma progression via miR-BART19-3p/APC/β-catenin axis.

Molecular cancer
2022

Onset of pulmonary Epstein-Barr virus-positive diffuse large B-cell lymphoma in a patient with silicosis.

Thoracic cancer
2022

Epstein-Barr virus-positive diffuse large B-cell lymphoma after frontline brentuximab vedotin treatment of classical Hodgkin lymphoma.

Annals of hematology
2021

A case report of right ventricular outflow tract obstruction caused by B-cell lymphoma: a rare presentation in an adult patient with pulmonary atresia.

European heart journal. Case reports
2022

A sporadic case of CTLA4 haploinsufficiency manifesting as Epstein-Barr virus-positive diffuse large B-cell lymphoma.

Journal of clinical and experimental hematopathology : JCEH
2021

Clinical and molecular features of Epstein-Barr virus-positive diffuse large B-cell lymphoma: Results in a multi-center trial.

Clinical and translational medicine
2022

Successful treatment of refractory retroperitoneal Epstein-Barr virus-positive diffuse large B-cell lymphoma with secondary hemophagocytic syndrome by sequential combination regimen of PD-1 blockade and chimeric antigen receptor T cells: a case report.

Anti-cancer drugs
2021

Secondary Hemophagocytic Lymphohistiocytosis With Epstein-Barr Virus-Associated Transformed Follicular Lymphoma: A Case Report and Literature Review.

Frontiers in oncology
2021

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

Clinical case reports
2021

Characteristics of Central Nervous System (CNS) Involvement in Children With Non-Hodgkin's Lymphoma (NHL) and the Diagnostic Value of CSF Flow Cytometry in CNS Positive Disease.

Technology in cancer research &amp; treatment
2021

Hemophagocytic lymphohistiocytosis associated with Epstein-Barr virus-positive diffuse large B-cell lymphoma, NOS of bone marrow-liver-spleen type: an autopsy case report.

Journal of clinical and experimental hematopathology : JCEH
2021

The Incidence of Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma: A Systematic Review and Meta-Analysis.

Cancers
2021

Epstein-Barr virus-positive diffuse large B-cell lymphoma with human immunodeficiency virus mimicking complicated frontal sinusitis: A case report.

World journal of clinical cases
2022

Cutaneous Epstein-Barr virus-positive diffuse large B-cell lymphoma, not otherwise specified: A systematic review and comparative analysis with Epstein-Barr virus-negative, leg type.

Journal of the American Academy of Dermatology
2020

Multiple cutaneous ulcers revealing a primary cutaneous Epstein-Barr virus-positive diffuse large B-cell lymphoma.

Clinical case reports
2020

EBV Positivity and Programmed Death-ligand 1 Expression in Diffuse Large B-cell Lymphoma: A Systematic Review.

Anticancer research
2020

T-cell lymphoma with a granulomatous lesion of the lungs after autologous hematopoietic stem cell transplantation for Epstein-Barr virus-positive diffuse large B-cell lymphoma: a unique rare case of metachronous B-cell and T-cell lymphoma.

Diagnostic pathology
2020

[Epstein-Barr virus positive diffuse large B-cell lymphoma in adolescents with sleep snoring as the first symptom: a case report].

Zhonghua er bi yan hou tou jing wai ke za zhi = Chinese journal of otorhinolaryngology head and neck surgery
2020

Infectious mononucleosis mimicking Epstein-Barr virus positive diffuse large B-cell lymphoma not otherwise specified.

International journal of hematologic oncology
2021

Clinicopathological analysis of neoplastic PD-L1-positive EBV+ diffuse large B cell lymphoma, not otherwise specified, in a Japanese cohort.

Virchows Archiv : an international journal of pathology
2020

[Analysis of Factors Influencing Clinical Efficacy of Rituximab on Patients with Epstein-Barr Virus Positive Diffuse Large B-Cell Lymphoma].

Zhongguo shi yan xue ye xue za zhi
2020

[Persistent malnutrition caused by Nihonkaiense diphyllobothriasis diagnosed during treatment of malignant lymphoma].

[Rinsho ketsueki] The Japanese journal of clinical hematology
2021

Epidermotropic Epstein-Barr virus-Positive Diffuse Large B-Cell Lymphoma: A Series of 3 Cases of a Very Unusual High-Grade Lymphoma.

The American Journal of dermatopathology
2021

Successful Rituximab Monotherapy in Advanced-stage, Epstein-Barr Virus-positive Diffuse Large B-Cell Lymphoma in an Adolescent With Systemic Juvenile Idiopathic Arthritis.

Journal of pediatric hematology/oncology
2020

[Composite adult T-cell leukemia/lymphoma and Epstein-Barr virus-positive diffuse large B-cell lymphoma].

[Rinsho ketsueki] The Japanese journal of clinical hematology
2020

A phase II study of ibrutinib in combination with rituximab-cyclophosphamide-doxorubicin hydrochloride-vincristine sulfate-prednisone therapy in Epstein-Barr virus-positive, diffuse large B cell lymphoma (54179060LYM2003: IVORY study): results of the final analysis.

Annals of hematology
2020

A Rare, Yet Treatable Pancreatic Tumor: Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma.

The American journal of case reports
2020

Epstein-Barr virus-positive diffuse large B-cell lymphoma features disrupted antigen capture/presentation and hijacked T-cell suppression.

Oncoimmunology
2020

Epstein-Barr virus-positive diffuse large B-cell lymphoma after sustained remission of T-cell prolymphocytic leukemia with alemtuzumab.

Leukemia &amp; lymphoma
2020

Impact of treatment variability and clinicopathological characteristics on survival in patients with Epstein-Barr-Virus positive diffuse large B cell lymphoma.

British journal of haematology
2019

Fatal Hypogammaglobulinemia 3 Years after Rituximab in a Patient with Immune Thrombocytopenia: An Underlying Genetic Predisposition?

Case reports in immunology
2019

pSTAT3 and MYC in Epstein-Barr virus-positive diffuse large B-cell lymphoma.

The Malaysian journal of pathology
2020

Epstein-Barr virus-positive diffuse large B cell lymphoma, not otherwise specified, carrying a t(19;22)(q13;q11) translocation.

Annals of hematology
2019

Primary cutaneous Epstein-Barr virus-positive diffuse large B-cell lymphoma (DLBCL) in a patient taking fingolimod.

Dermatology online journal
2019

Expression of co-inhibitory molecules B7-H4 and B7-H1 in Epstein-Barr virus positive diffuse large B-cell lymphoma and their roles in tumor invasion.

Pathology, research and practice
2020

Primary cutaneous Epstein-Barr virus-positive diffuse large B-cell lymphoma, not otherwise specified, in a nonimmunocompromised young man: A case report.

Journal of cutaneous pathology
2020

Multiple Brain Biopsies for Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma with Extensive Necrosis in a Posttransplant Patient.

World neurosurgery
2019

B7-H4 is highly expressed in aggressive Epstein-Barr virus positive diffuse large B-cell lymphoma and inhibits apoptosis through upregulating Erk1/2 and Akt signalling pathways.

Infectious agents and cancer
2019

[Prevalence and clinico-pathological characteristics in patients with Epstein-Barr virus-positive diffuse large B-cell lymphoma in a high complexity hospital in Cali, Columbia].

Revista espanola de patologia : publicacion oficial de la Sociedad Espanola de Anatomia Patologica y de la Sociedad Espanola de Citologia
2019

The tumour microenvironment is immuno-tolerogenic and a principal determinant of patient outcome in EBV-positive diffuse large B-cell lymphoma.

European journal of haematology
2019

[Genetic Alterations Involving PD-L1/PD-L2 in Human Malignancies].

Gan to kagaku ryoho. Cancer &amp; chemotherapy
2019

Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma, Not Otherwise Specified, in the Oral Cavity.

The Journal of craniofacial surgery
2018

Primary cutaneous Epstein-Barr virus-positive diffuse large B-cell lymphoma: a rare and aggressive cutaneous lymphoma.

Cutis
2019

LOCALLY INVASIVE PHEOCHROMOCYTOMA COMBINED WITH PRIMARY MALIGNANT ADRENAL LYMPHOMA.

AACE clinical case reports
2018

Genetic heterogeneity and mutational signature in Chinese Epstein-Barr virus-positive diffuse large B-cell lymphoma.

PloS one
2018

Different prevalence and clinical outcome of Epstein-Barr virus positive diffuse large B-cell lymphoma between North American and non-Western populations.

Annals of translational medicine
2018

The Diving Bell and the Butterfly Revisited: A Fatal Case of Locked-in Syndrome in a Man With Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma, Not Otherwise Specified.

Clinical medicine insights. Blood disorders
2018

Outcomes among North American patients with diffuse large B-cell lymphoma are independent of tumor Epstein-Barr virus positivity or immunosuppression.

Haematologica
2017

Pediatric Pulmonary Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma: A Case Report and Review of the Literature.

Case reports in pathology
2018

Epstein-Barr virus positive diffuse large B-cell lymphoma presenting with vaginal sloughing and ulcerated skin nodule.

Journal of cutaneous pathology
2018

Epstein-Barr virus positive diffuse large B cell lymphoma with G17V RHOA mutation arising from peripheral T cell lymphoma, not otherwise specified.

Annals of hematology
2018

Tocilizumab for severe cytokine-release syndrome after haploidentical donor transplantation in a patient with refractory Epstein-Barr virus-positive diffuse large B-cell lymphoma.

Hematological oncology
2017

Epstein-Barr virus-positive diffuse large B-cell lymphoma.

Proceedings (Baylor University. Medical Center)
2017

Epstein-Barr virus (EBV) provides survival factors to EBV+ diffuse large B-cell lymphoma (DLBCL) lines and modulates cytokine induced specific chemotaxis in EBV+  DLBCL.

Immunology
2017

Frequent downregulation of BTB and CNC homology 2 expression in Epstein-Barr virus-positive diffuse large B-cell lymphoma.

Cancer science
2017

Synchronous Microscopic Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma of the Adrenal and Lymphoplasmacytic Lymphoma: De Novo Disease or Transformation?

International journal of surgical pathology
2016

Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma Involving the Colon in a Patient With Ulcerative Pancolitis and Polymyositis on Long-Term Methotrexate Therapy.

Gastroenterology research
2016

Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma: Is It Different between Over and Under 50 Years of Age?

Asian Pacific journal of cancer prevention : APJCP
2016

Epstein-Barr virus-positive diffuse large B cell lymphoma arising from a chronic lymphocytic leukemia: Overlapping features with classical Hodgkin lymphoma.

Pathology international
2016

Next generation sequencing revealed DNA ligase IV deficiency in a "developmentally normal" patient with massive brain Epstein-Barr virus-positive diffuse large B-cell lymphoma.

Clinical immunology (Orlando, Fla.)
2015

[RS3PE syndrome associated with senile Epstein-Barr virus-positive diffuse large B cell lymphoma of a patient with colon cancer].

[Rinsho ketsueki] The Japanese journal of clinical hematology
2015

[Clinicopathological features and prognositic analysis of Epstein-Barr virus- positive diffuse large B-cell lymphoma in elderly: 7 cases report and literatures review].

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi
2015

PD-1 Blockade Can Restore Functions of T-Cells in Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma In Vitro.

PloS one
2015

Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly: a concise review and update.

Current opinion in oncology
2015

Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma in the Elderly: A Matched Case-Control Analysis.

PloS one
2015

Epstein-Barr virus positive diffuse large B-cell lymphoma predict poor outcome, regardless of the age.

Scientific reports
2015

Age cutoff for Epstein-Barr virus-positive diffuse large B-cell lymphoma--is it necessary?

Oncotarget
2016

Epstein-Barr Virus-Positive Diffuse Large B cell Lymphoma in the Experience of a Tertiary Medical Center in Poland.

Archivum immunologiae et therapiae experimentalis
2015

EBV+ diffuse large B-cell lymphoma arising within atrial myxoma in Chinese immunocompetent patient.

International journal of clinical and experimental pathology
2015

Epstein-Barr virus-positive diffuse large B-cell lymphoma following acute myeloid leukemia: a common clonal origin indicated by chromosomal translocation t(3;4)(p25;q21).

International journal of hematology
2015

[Updates on Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly].

Zhonghua bing li xue za zhi = Chinese journal of pathology
2015

Integrated copy number and gene expression profiling analysis of Epstein-Barr virus-positive diffuse large B-cell lymphoma.

Genes, chromosomes &amp; cancer
2015

Epstein-Barr virus-positive diffuse large B-cell lymphoma in children: a disease reminiscent of Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly.

Human pathology
Ver todos os 125 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

Ainda não temos associações cadastradas para Linfoma B difuso de grandes células positivo para o vírus Epstein-Barr.

É de uma associação que acompanha esta doença? Fale com a gente →

Doença com base genética

Um médico geneticista pode ajudar no diagnóstico de Linfoma B difuso de grandes células positivo para o vírus Epstein-Barr e no aconselhamento genético da família.

Vai consultar um especialista? Confirme o registro dele no conselho.
Conselhos e sociedades

Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Ainda não achamos doenças com sintomas parecidos o suficiente.

Perguntas frequentes

O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu

Respostas geradas por IA a partir das fontes citadas

Não, o padrão de herança familiar desta doença é classificado como não aplicável nos catálogos médicos. Ela se desenvolve de forma adquirida ao longo da vida e não é transmitida geneticamente aos filhos.

Referências

Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete

4 fontes citadas no texto · 6 publicações do grafo RarasNet (PubMed) · 4 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.

  1. MONDO
    purl.obolibrary.org
  2. GARD:21150
    GARD (NIH)
  3. Q25112638
    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.

Citar este verbete

Raras. (2026). Linfoma B difuso de grandes células positivo para o vírus Epstein-Barr. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/linfoma-b-difuso-de-grandes-celulas-positivo-para-o-virus-epstein-barr

Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Compêndio · Raras BR

Linfoma B difuso de grandes células positivo para o vírus Epstein-Barr

ORPHA:289661 · MONDO:0017346
Prevalência
Unknown
Herança
Not applicable
CID-10
C83.3 · Linfoma não-Hodgkin difuso, grandes células (difuso)
CID-11
Ensaios
2 ativos
Início
Adult, Elderly
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C2700007
EuropePMC
Wikidata
Papers 10a

📋 Origem dos dados

Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.

Doença rara (ontologia)
fonte: Orphanet
Identificador unificado
fonte: MONDO
Dado público estruturado
fonte: Wikidata
Rara