É um grupo de doenças caracterizadas pela multiplicação descontrolada de células B, T ou NK (tipos de células de defesa), que foram infectadas pelo vírus Epstein-Barr. A forma como a doença se manifesta e sua gravidade dependem da imunidade da pessoa e de fatores do próprio vírus. A persistência viral nessas células pode causar desde um crescimento celular simples e reativo até tipos agressivos de câncer como linfomas e leucemias, cada um com características clínicas e patológicas únicas.
Introdução
O que você precisa saber de cara
É um grupo de doenças caracterizadas pela multiplicação descontrolada de células B, T ou NK (tipos de células de defesa), que foram infectadas pelo vírus Epstein-Barr. A forma como a doença se manifesta e sua gravidade dependem da imunidade da pessoa e de fatores do próprio vírus. A persistência viral nessas células pode causar desde um crescimento celular simples e reativo até tipos agressivos de câncer como linfomas e leucemias, cada um com características clínicas e patológicas únicas.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 20 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 57 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
2 genes identificados com associação a esta condição.
Transcription factor that binds DNA in a non-specific manner, yet also specifically recognizes the core sequence 5'-CAC[GA]TG-3' (PubMed:24940000, PubMed:25956029). Activates the transcription of growth-related genes (PubMed:24940000, PubMed:25956029). Binds to the VEGFA promoter, promoting VEGFA production and subsequent sprouting angiogenesis (PubMed:24940000, PubMed:25956029). Regulator of somatic reprogramming, controls self-renewal of embryonic stem cells (By similarity). Functions with TAF
Nucleus, nucleoplasmNucleus, nucleolusNucleusCytoplasmChromosome
Involved in pinching off the separated nuclei at the cleavage furrow and in cytokinesis (PubMed:20107318). Required for mitotic integrity and maintenance of chromosomal stability. Protects cells against mitotic errors, centrosomal amplification, micronucleus formation and aneuploidy. Plays a key role of midbody function involving abscission of the daughter cells during cytokinesis and appropriate chromosomal and nuclear segregation into the daughter cells (PubMed:22988245, PubMed:23713010)
CytoplasmMidbody
Lymphoma, Hodgkin, classic
A malignant disease characterized by progressive enlargement of the lymph nodes, spleen and general lymphoid tissue, and the presence of large, usually multinucleate, cells (Reed-Sternberg cells). Reed-Sternberg cells compose only 1-2% of the total tumor cell mass. The remainder is composed of a variety of reactive, mixed inflammatory cells consisting of lymphocytes, plasma cells, neutrophils, eosinophils and histiocytes.
Medicamentos e terapias
Mecanismo: Tubulin inhibitor
Mecanismo: Programmed cell death protein 1 inhibitor
Mecanismo: DNA topoisomerase II alpha inhibitor
Mecanismo: Tubulin inhibitor
Variantes genéticas (ClinVar)
80 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
19 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Doença linfoproliferativa associada a vírus Epstein-Barr
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Ensaios clínicos abertos e novidades científicas recentes
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Publicações mais relevantes
Molecular pathogenesis and evolving treatment landscape of extranodal NK/T-cell lymphoma.
Extranodal NK/T-cell lymphoma (ENKTL) is a rare, aggressive Epstein-Barr virus (EBV)-associated malignancy. The 5th edition of the WHO classification refined their nomenclature by removing "nasal type" and introducing related entities to reflect updated biological insights. This review synthesizes recent advances in epidemiology and molecular pathogenesis in ENKTL, highlighting recurrent driver gene alterations, particularly frequent mutations in X-linked genes such as DDX3X and MSN, alongside TP53 mutations and PD-L1 structural variations that facilitate immune evasion. We also discuss novel mouse models that replicate the ENKTL niche, revealing how Trp53 loss and the EBV oncoprotein LMP1 synergistically remodel the tumor microenvironment to promote lymphomagenesis. Therapeutically, while L-asparaginase-based regimens including SMILE and P-GemOx remain the standard of care, emerging immune checkpoint inhibitors have demonstrated encouraging efficacy, particularly in combination with chemotherapy. This review provides an integrated perspective on the evolving landscape of diagnosis, disease biology, and translational treatment strategies for NK/T-cell lymphoproliferative disorders.
Epstein-Barr virus infection in children with liver transplantation.
Epstein-Barr virus (EBV) infection commonly occurs in children, particularly in developing countries. Most children infected with EBV are asymptomatic, though some develop significant complications, including EBV-associated malignancies, hemophagocytic lymphohistiocytosis, and multiple sclerosis. In immunocompromised children, including those with liver transplantation, EBV infection manifests with a diverse spectrum of presentations, varying from asymptomatic to post-transplant lymphoproliferative disorder, which can evolve into lymphoma. Therefore, close monitoring, early detection, and prompt management are crucial. This review aimed to investigate the pathogenesis and manifestations of EBV in healthy children and those who underwent liver transplantation. The proposed algorithm for early EBV detection and management, along with case studies, is provided to help pediatricians increase their index of suspicion.
Classic hodgkin lymphoma associated with Epstein-Barr virus reactivation during vedolizumab therapy for ulcerative colitis.
Epstein-Barr virus (EBV)-associated lymphoproliferative disorders have been reported in patients receiving immunosuppressive therapy for inflammatory bowel disease; however, the onset of classic Hodgkin lymphoma (cHL) during vedolizumab therapy is extremely rare. A 73 year-old woman with ulcerative colitis (UC) was steroid-dependent, showing disease exacerbation when prednisolone was tapered or discontinued. Azathioprine was administered eight years prior but was discontinued after a short period due to liver dysfunction. Vedolizumab was initiated 31 months before presentation. Despite initial improvements in UC with vedolizumab, hematochezia subsequently persisted. Other biologics or immunomodulators that could cause systemic immunosuppression were avoided because she was receiving treatment for breast cancer. Colonoscopy showed erosions in the transverse colon and an ulcerative lesion in the sigmoid colon. A biopsy revealed high-grade dysplasia in the transverse colon and inflammatory granulation tissue without malignancy in the sigmoid lesion; therefore, surgery was indicated. Laparoscopic total proctocolectomy was performed, and the surgical specimen contained Reed-Sternberg cells positive for CD20, CD30, PD-L1, and EBV-encoded RNA, establishing EBV-associated cHL. Although the development of cHL is likely multifactorial, clinicians should be aware that EBV-associated intestinal cHL may develop in patients with UC receiving vedolizumab when tumor-like ulcers or mural thickening are encountered.
A multidimensional workflow profiling of allogeneic virus-specific T cell therapies reveals potency-linked signatures.
Allogeneic virus-specific T cell (VST) therapies offer distinct advantages, including scalability, rapid deployment, and manufacturing consistency, and have demonstrated efficacy in multiple clinical trials. However, identifying VST products with high therapeutic potential remains a major hurdle. Here, we present a multidimensional analytical platform that integrates in vitro and in vivo anti-viral reactivity, T cell receptor (TCR) repertoire analysis, gene expression profiling, immunophenotyping, and functional validation in a humanized mouse model. Epstein-Barr virus (EBV)-specific T cells expanded from HLA-diverse healthy donors consistently enriched for TCRs targeting EBV-encoded antigens. Transcriptomic and high-dimensional flow cytometric analyses revealed a distinct effector-associated signature. Importantly, this integrative approach uncovered correlative biomarkers of T cell potency and effector function, validated in an in vivo model of EBV-driven B cell lymphoma. These findings establish a scalable framework for the characterization of allogeneic T cell products and may inform the development of predictive metrics for in vivo efficacy.
Withaferin A inhibits EBV-driven lymphomagenesis through multiple mechanisms, including EBNA1 degradation.
Epstein-Barr virus (EBV) infects over 90% of the global population and drives multiple aggressive B-cell malignancies, including Burkitt lymphoma, diffuse large B-cell lymphoma, and Hodgkin lymphoma. Standard chemoimmunotherapy regimens can be highly effective, yet Epstein-Barr virus positive (EBV+) lymphomas sometimes exhibit poorer responses, higher resistance, and worse survival compared with Epstein-Barr virus-negative (EBV-) counterparts. This reflects the virus's ability to drive immune evasion, alter cell death pathways, and exploit host immune dysfunction, underscoring the potential value of EBV-directed strategies. Withaferin A (WA), a steroidal lactone with known anticancer and anti-inflammatory properties, was evaluated for its efficacy against EBV-associated B-cell non-Hodgkin lymphomas (B-NHL). Across a panel of lymphoma cell lines, WA demonstrated selective cytotoxicity toward EBV+ B-NHL, in part through proteasome-dependent degradation of EBNA1 (EBV nuclear antigen 1) and subsequent loss of viral episomes, alongside additional effects on cellular stress and survival pathways. Mechanistic studies revealed that WA collapses antioxidant defenses, drives oxidative stress, and suppresses NF-κB signaling, creating a multipronged disruption of viral and host survival pathways. In primary B-cell models and a cord blood-humanized mouse model of EBV-driven lymphomagenesis, WA inhibited B-cell transformation, reduced splenomegaly and tumor burden, and significantly prolonged survival without evidence of increased viral replication. These findings establish WA as a potent preclinical candidate that selectively targets vulnerabilities unique to EBV-transformed B cells, supporting further optimization and evaluation for EBV+ B-cell malignancies.
Publicações recentes
[Clinical Characteristics and Prognostic Analysis of Patients with EBV-Positive Diffuse Large B-Cell Lymphoma].
[EBNA1 Knockdown Modulates Proliferation, Apoptosis, and Metabolism in Burkitt's Lymphoma Cells by Downregulating miR-127-5p].
A 43-Year-Old Man With Isolated Cutaneous Lymphomatoid Granulomatosis Presenting as a Chronic Necrotizing Ulcer of the Upper Arm.
Composite lymphoma composed of follicular lymphoma and nodal T-follicular helper cell lymphoma: report of 3 cases highlighting histopathologic zonation of each neoplastic component.
Temporal association of diffuse large B-cell lymphoma with PD-1 inhibitor therapy in a patient with gastric adenocarcinoma: a case report.
📚 EuropePMCmostrando 199
[Clinical Characteristics and Prognostic Analysis of Patients with EBV-Positive Diffuse Large B-Cell Lymphoma].
Zhongguo shi yan xue ye xue za zhi[EBNA1 Knockdown Modulates Proliferation, Apoptosis, and Metabolism in Burkitt's Lymphoma Cells by Downregulating miR-127-5p].
Zhongguo shi yan xue ye xue za zhiA 43-Year-Old Man With Isolated Cutaneous Lymphomatoid Granulomatosis Presenting as a Chronic Necrotizing Ulcer of the Upper Arm.
The American journal of case reportsMolecular pathogenesis and evolving treatment landscape of extranodal NK/T-cell lymphoma.
Proceedings of the Japan Academy. Series B, Physical and biological sciencesEpstein-Barr virus infection in children with liver transplantation.
World journal of hepatologyComposite lymphoma composed of follicular lymphoma and nodal T-follicular helper cell lymphoma: report of 3 cases highlighting histopathologic zonation of each neoplastic component.
American journal of clinical pathologyTemporal association of diffuse large B-cell lymphoma with PD-1 inhibitor therapy in a patient with gastric adenocarcinoma: a case report.
Frontiers in immunologyClassic hodgkin lymphoma associated with Epstein-Barr virus reactivation during vedolizumab therapy for ulcerative colitis.
Clinical journal of gastroenterologyExpression of MicroRNA-155 and its associations with EBV serological markers and inflammatory cytokines in young lymphoma patients with evidence of active EBV infection.
Experimental biology and medicine (Maywood, N.J.)Inhibition of death-ligand-induced apoptosis in Epstein-Barr virus-encoded small RNAs-expressing human T-cell line.
Infectious agents and cancerMachine Learning Models for Posttransplant Lymphoproliferative Disorder (PTLD) Risk Prediction in Thoracic Transplantation.
ASAIO journal (American Society for Artificial Internal Organs : 1992)Rapidly Expanded EBV-Specific T Cells for the Treatment of Refractory EBV Reactivation and EBV-Related Lymphoproliferative Disorders.
Open forum infectious diseasesHemophagocytic lymphohistiocytosis associated with extranodal NK/T cell lymphoma, nasal type or aggressive NK cell leukemia: a retrospective multicenter study of Jiangsu Cooperative Lymphoma Group (JCLG).
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MedicineBilateral Breast Implant-Associated Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma: A Case Report and Literature Review.
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Frontiers in immunologyClassical Hodgkin lymphoma associated with immune deficiency and dysregulation exhibits a prognosis similar to sporadic cases but with distinct predictive markers.
Annals of hematologyDiagnostic challenges in Burkitt's lymphoma with skull base and paravertebral involvement, and hearing loss: a case report.
Journal of medical case reportsGermline Homozygous RAG1 Missense Variant Associated With Epstein-Barr Virus Negative Childhood Burkitt Lymphoma: A Case Report.
Journal of pediatric hematology/oncologyEpstein-Barr Virus-Associated T/NK-Cell Neoplasms.
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Nature communicationsHigher chemical complementarity of EBV epitopes and IGH CDR3s correlates with better outcomes for lymphoma and ovarian cancer.
Cancer causes & control : CCCDissecting Epstein-Barr Virus Dependence Across Diverse Infected Cell Models.
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Pathology, research and practiceHistological subtypes of rheumatoid arthritis-associated lymphoproliferative disorders: Serological and Epstein-Barr virus findings.
Modern rheumatologyClassic Hodgkin lymphoma in a patient with Behçet's disease: a case report and review of the literature.
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Journal of medical virologyHIV-associated non-Hodgkin lymphoma tumor-microenvironment axes differ by EBV status across cellular origins.
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Annals of hematologyRecent advances in infections as risk factors for lymphoma: a review of the effects of viral, bacterial, and fungal infections in lymphoma.
Archives of microbiologyMonocyte/macrophages may exert prominent roles in macrophage activation syndrome.
Annals of hematology[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 hematologyLysine-specific histone demethylase complex restricts Epstein-Barr virus lytic reactivation.
Nature microbiologyPrimary Cutaneous B-Cell Lymphomas: An Updated Portrait of Classification, Biology, and Clinical Management.
European journal of haematology[Primary cutaneous B-cell lymphomas-an overview of established and novel aspects].
Dermatologie (Heidelberg, Germany)Epstein-Barr Virus-Positive Lymphoproliferative Disorder in an HIV Patient Successfully Treated With Rituximab.
CureusLMP2A-Targeting CAR-T Cells Equipped With Inducible IL-18 to Address EBV-Associated Malignancies.
HLAWithaferin A inhibits EBV-driven lymphomagenesis through multiple mechanisms, including EBNA1 degradation.
BloodBurkitt in disguise: clonal transformation of incidentally detected gallbladder diffuse large B-cell lymphoma in a liver transplant recipient.
International journal of hematologyOral Plasmablastic Lymphoma: Case Series and Literature Review.
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Virologica SinicaAflatoxin B1 induces subtle but coordinated histone modifications in Epstein-Barr virus infected and non-infected Burkitt lymphoma cells.
Environment internationalDiagnostic Challenges in Diffuse Large B-Cell Lymphoma in a Young Liver Transplant Patient with Ulcerative Colitis: A Case Report.
The American journal of case reportsThe incidence and role of EBV and HIV in head and neck lymphomas: an institutional study.
Oral surgery, oral medicine, oral pathology and oral radiologyPediatric Liver Transplant Complications: EBV-Associated Tumors and Infection Management Strategies.
The American journal of case reports[Monomorphic epitheliotropic intestinal T-cell lymphoma: a clinicopathological and genetic mutation characteristics analysis of forty-two cases].
Zhonghua bing li xue za zhi = Chinese journal of pathology[New advances in the pathology and diagnostic challenges of extranodal NK/T-cell lymphoma].
Zhonghua bing li xue za zhi = Chinese journal of pathology[Current status and challenges of AIDS-related malignant lymphoma].
[Rinsho ketsueki] The Japanese journal of clinical hematologyMethotrexate-Associated Primary Cutaneous Epstein-Barr Virus-Positive Polymorphic B-Cell Lymphoproliferative Disorder With Metachronous T-Cell Clonally Related Angioimmunoblastic T-Cell Lymphoma: A Case Report and Review of the Literature.
The American Journal of dermatopathologyThe clinical features and outcomes of aggressive large B cell lymphoma with concomitant hemophagocytic lymphohistiocytosis at diagnosis.
Annals of hematologyCD20-Negative Large B-Cell Lymphomas: The Diagnostic Challenge of Tumors with Downregulation of Mature B-Cell Marker Expression.
International journal of molecular sciencesFollicular Helper T-Cell Lymphomas With Epstein-Barr Virus-Positive Neoplastic Cells: A Rare Scenario With Diagnostic Implications.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncLong-Term Efficacy of Epstein-Barr Virus-Specific T Cells for Epstein-Barr Virus-Associated Post-Transplantation Lymphoproliferative Disorder after Allogeneic Stem Cell Transplantation: A Real-World Study.
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BMC nephrologyClinical and functional characterization of a novel TNFRSF9 variant causing immune dysregulation with predisposition to EBV-driven lymphomagenesis.
Frontiers in immunologyIncidence and outcomes of post-transplant lymphoproliferative disorder in the Western Australian liver transplant population: a 24-year retrospective study.
Internal medicine journalPeripheral EBV antigen-specific T cell is dysfunctional in Epstein-Barr virus positive diffuse large B-cell lymphoma.
BMC cancerClinical Significance of Plasma and Peripheral Blood Mononuclear Cell EBV-DNA in Lymphoma.
Journal of clinical laboratory analysisPrimary Immunodeficiencies and EBV-Associated Diseases.
Current topics in microbiology and immunologyASXL1 deficiency causes epigenetic dysfunction, combined immunodeficiency, and EBV-associated lymphoma.
The Journal of experimental medicineCurrent State of AIDS-Related Malignant Lymphoma.
VirusesClinical characteristics and prognosis of EBV-Positive mature B-Cell lymphoma in children: a retrospective study.
Annals of hematologyThe expanding spectrum of T-cell lymphomas with follicular helper T-cell phenotype and implications for differential diagnoses: A report of the 2023 SH/EA4HP Lymphoma Workshop.
American journal of clinical pathologyA genome-wide association study identifies an African-specific locus on chromosome 21q22.12 associated with Burkitt lymphoma risk and survival.
LeukemiaExploring the Genetic Landscape of Primary Marginal Zone Lymphoma of the Urinary Bladder.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncCase Report: dose adjustment of orelabrutinib for managing central nervous system post-transplant lymphoproliferative disorder following acute lymphoblastic leukemia transplantation.
Frontiers in immunologyCrescentic glomerulonephritis associated with NK-large granular lymphocytic leukemia: A case report.
MedicineEpstein-Barr virus-infected tonsillar marginal zone B cells in vivo as a precursor for immunosuppression-related B-cell lymphoma.
Journal of virologyPARP1 Inhibition Halts EBV+ Lymphoma Progression by Disrupting the EBNA2/MYC Axis.
Journal of medical virologyEpstein-Barr virus-positive T- and NK-cell lymphoproliferative disorders: Report from the 2023 SH/EA4HP Lymphoma Workshop.
American journal of clinical pathologyEpstein Barr virus antibody and cancer risk in two prospective cohorts in Southern China.
Nature communicationsPlasmablastic Lymphoma-A Crosstalk Between Myeloma and Lymphoma.
Clinical lymphoma, myeloma & leukemiaTCF3 and ID3 Regulate TSPAN32 Expression in Burkitt Lymphoma.
Scandinavian journal of immunologyAssociation of Epstein-Barr virus genomic alterations with human pathologies.
BloodHigh PD-L1 Expression in HRS Cells and Macrophages in Tumor Immune Microenvironment Is Associated with Adverse Outcome and EBV Positivity in Classical Hodgkin Lymphoma.
International journal of molecular sciencesProtracted Tonsillitis as an Atypical Initial Manifestation of Methotrexate-Induced EBV-Positive Lymphoproliferative Disorder in Rheumatoid Arthritis: A Case Report and Literature Review.
Diagnostics (Basel, Switzerland)Kaposi sarcoma-associated herpesvirus cooperates with Epstein-Barr virus to co-transform a small set of human B cells oncogenically.
PLoS pathogensImaging of Immunodeficiency-Associated Lymphoproliferative Disorders.
Seminars in ultrasound, CT, and MRMethotrexate-associated Angioimmunoblastic T-cell Lymphoma Presenting with Acute Kidney Injury.
Internal medicine (Tokyo, Japan)Integrative Genomic and Transcriptomic Analysis Reveals Targetable Vulnerabilities in Angioimmunoblastic T-Cell Lymphoma.
American journal of hematology[Methotrexate-related lymphoproliferative disease of the ileum with fistular formation between the small bowel and rectum:a case report].
Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterologyExtramedullary plasmacytoma in children is a genetically distinct localized neoplasia curable by surgical resection.
Blood advancesTumor-Associated Sympathetic Nerves Promote the Progression of Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma.
Advanced science (Weinheim, Baden-Wurttemberg, Germany)Prophylactic and Therapeutic EBV Vaccination.
Current topics in microbiology and immunologyEfficacy and prognosis of allogeneic hematopoietic stem cell transplantation in aggressive NK-cell leukemia: a meta-analysis.
Annals of hematologyDifferential expression of proliferation and immune response genes between children and adults influences survival of diffuse large B cell lymphoma.
Scientific reportsGenetic Profiling Reveals the Distinctions Among MTX-Associated DLBCL, EBV-Positive Mucocutaneous Ulcer, and EBV + DLBCL.
Cancer scienceClinicopathological features and prognostic factors of AIDS-related lymphoma: a retrospective single-center study in China.
Annals of hematologyInternalization of extracellular double-stranded DNA as a potential marker of cancer stem cells in Epstein-Barr virus-induced B-cell lymphoma.
Cancer biomarkers : section A of Disease markersEpstein-Barr virus-associated infectious mononucleosis exhibits substantially higher non-treponemal test titers in biological false-positive reactions.
Journal of infection and chemotherapy : official journal of the Japan Society of ChemotherapyEBV-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 immunologyHarnessing Cellular Immunotherapy for EBV-Associated Malignancies: Current Advances and Future Directions.
Journal of cellular and molecular medicineTargeting intracellular LMP2 with costimulatory signal-armed antibody-like TCR T cells.
JCI insightEpstein-Barr virus latent membrane protein 1 subverts IMPDH pathways to drive B-cell oncometabolism.
PLoS pathogensMicroRNA changes with macro potential contribute to secondary immunodeficiency in chronic lymphocytic leukemia during epstein barr virus reactivation.
Scientific reportsSTAT3, MYC, and EBNA1 cooperate through a ZC3H18 transcriptional network to regulate survival and proliferation of EBV-positive lymphomas.
PLoS pathogensDNA Methylation Epitypes of Burkitt Lymphoma with Distinct Molecular and Clinical Features.
Blood cancer discoverySpatial transcriptomics reveals tumor microenvironment heterogeneity in EBV positive diffuse large B cell lymphoma.
Scientific reportsLeveraging the interconnected unfolded protein response and NLRP3 inflammasome pathways to reactivate Epstein-Barr virus in diffuse large B-cell lymphomas.
NAR cancerIntegration of anti-PD-1 antibody into chemotherapeutic regimens improved the outcome of aggressive NK cell leukemia: a single-center retrospective real-world analysis.
Frontiers in immunologySystemic EBV+ T-Cell Lymphoma of Childhood with Hemophagocytic Lymphohistiocytosis in a Patient with a Highly Complex Karyotype.
GenesHuman Immune Responses to Epstein-Barr Virus Highlighted by Immunodeficiencies.
Annual review of immunologyUnderstanding EBV infection and EBV-associated lymphomas in children.
VirologyLong-term outcomes in patients with persistent EBV-DNA positivity after primary Epstein-Barr virus infection and clinical implications of rituximab therapy.
International immunopharmacologyConcomitant Splenic Tuberculosis and Epstein-Barr Virus-Related T-Cell Leukemia/Lymphoma in a 28-Year-Old Pregnant Woman in South Sudan.
The American journal of tropical medicine and hygieneCytogenomic and Clinicopathologic Comparison of MYC-Positive and MYC-Negative High-Grade B-Cell Lymphoma With 11q Aberration in the Context of Other Aggressive Lymphomas With MYC Rearrangement.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncFibrin-associated large B-cell lymphoma arising in a cystic lymphangiomatous lesion of the adrenal gland: A case report and overview of the entity.
Pathology, research and practicePlasmablastic lymphoma with cutaneous manifestations in an immunocompetent man: A rare case report with literature review.
Journal of cancer research and therapeuticsSubtyping Burkitt Lymphoma by DNA Methylation.
Genes, chromosomes & cancerComparison of nanopore with illumina whole genome assemblies of the Epstein-Barr virus in Burkitt lymphoma.
Scientific reports[Clinicopathological features and prognostic value of CD30 expression in EBV-positive diffuse large B cell lymphoma].
Zhonghua bing li xue za zhi = Chinese journal of pathologyA patient-derived T cell lymphoma biorepository uncovers pathogenetic mechanisms and host-related therapeutic vulnerabilities.
Cell reports. MedicineRecent advances in animal models of lymphomagenesis caused by human γ-herpesviruses.
Current opinion in virologyPandemic-Driven Shifts in Epstein-Barr Virus (EBV) Epidemiology: Single Center Study.
VirusesBurkitt lymphoma in patients with chronic lymphocytic leukemia and other indolent B-cell lymphoid malignancies.
Leukemia & lymphomaPrognostic impact of pre-treatment and post-treatment plasma Epstein-Barr virus DNA in peripheral T-cell lymphomas.
Annals of medicineHIV-negative primary effusion lymphoma: a series of seven cases.
HistopathologyDoes CD47 expression have prognostic significance in classical Hodgkin lymphoma?
Turkish journal of medical sciencesReal-world evidence regarding cancer, mortality, and graft failure risk with de novo belatacept use among kidney transplant recipients in the United States.
American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant SurgeonsLarge B-cell lymphoma associated with fluid overload: two new case reports with molecular insights.
Virchows Archiv : an international journal of pathologyIncorporation of Epstein-Barr viral variation implicates significance of Latent Membrane Protein 1 in survival prediction and prognostic subgrouping in Burkitt lymphoma.
International journal of cancerDevelopment and validation of a predictive model for diagnosing EBER-positive lymphoma-associated hemophagocytic lymphohistiocytosis.
BMC cancerNavigating Epstein-Barr Virus (EBV) and Post-Transplant Lymphoproliferative Disorder (PTLD) in Pediatric Liver Transplantation: Current Knowledge and Strategies for Treatment and Surveillance.
VirusesGenetic evidence identifies a causal relationship between EBV infection and multiple myeloma risk.
Scientific reportsPD-L1/PD-L2 genetic profile in the molecular cytogenetic classification of classic Hodgkin lymphoma.
Virchows Archiv : an international journal of pathologyDevelopment of a novel prognostic nomogram for AIDS-associated diffuse large B-cell lymphoma: a retrospective study from northern China.
Clinical and experimental medicineGp350-targeted CAR-T therapy in EBV-positive Burkitt lymphoma: pre-clinical development of gp350 CAR-T.
Journal of translational medicineAge-related immunosenescence and Epstein-Barr virus-positive mucocutaneous ulcer presenting as oral cancer: a case report.
Frontiers in medicineEpstein-Barr Virus-Encoded Latent Membrane Protein 2A Promotes Immune Escape by Upregulating SYK/Nuclear Factor-κB Signaling in Diffuse Large B-cell Lymphoma.
Laboratory investigation; a journal of technical methods and pathologyDifferential antibody response to EBV proteome following EBVST immunotherapy in EBV-associated lymphomas.
Blood advancesClassic Hodgkin lymphoma: Pathobiological features that impact emerging therapies.
Blood reviewsRisk and prognosis of posttransplant lymphoproliferative disease in Epstein-Barr virus-seronegative kidney transplant recipients - an observational cohort study from Norway and western Denmark.
American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant SurgeonsComputational pathology identifies a low B-cell content in the tumour microenvironment as a predictor of adverse outcome in patients with classic Hodgkin lymphoma treated with ABVD.
Journal of clinical pathologyBetter Outcomes for Ovarian Cancer Associated With the Detection of Anti-EBV TCR CDR3s: Potential Relevance to Diffuse Large B-Cell Lymphoma.
American journal of reproductive immunology (New York, N.Y. : 1989)Topological importance of CD8+ T-cell enrichment in the tumor microenvironment of classic Hodgkin lymphoma.
Annals of hematologyClinical, Pathologic, and Molecular spectrum of Angioinmmunoblastic T-cell Lymphoma Cutaneous Lesions: Clinical, Pathologic, and Molecular Analysis.
The American journal of surgical pathologyTargeted Next-Generation Sequencing of Cell-Free DNA to Detect MYC-Immunoglobulin Translocation and Epstein-Barr Virus DNA in Plasma of Burkitt Lymphoma Patients in East Africa.
JCO global oncologyRare Cutaneous Primary Presentation of Extracavitary Primary Effusion Lymphoma.
The American Journal of dermatopathologyRASGRP1 Deficiency Associated with Diffuse Mesangial Sclerosis Infantile Nephrotic Syndrome and Epstein-Barr Virus-Induced Hodgkin's Lymphoma.
Pediatric allergy, immunology, and pulmonologyPrimary thyroid B-cell lymphoma: molecular insights into its clonal evolution and relapse.
The Journal of pathologyEpstein-Barr virus-associated lymphoproliferative disease during remission after induction therapy with dasatinib in Philadelphia chromosome-positive acute lymphoblastic leukemia: a case report.
Annals of hematologySpontaneous Regression of Plasmablastic Lymphoma Associated With Methotrexate After Withdrawal.
Journal of hematologyRole of rapidly evolving immunotherapy in chronic active Epstein-Barr virus disease.
Frontiers in immunologyAtypical presentations of primary lymphoproliferative processes in the central nervous system.
RadiologiaMulti-modal analysis reveals tumor and immune features distinguishing EBV-positive and EBV-negative post-transplant lymphoproliferative disorders.
Cell reports. MedicineTransmission of an Epstein-Barr Strain Common in South-East China to a Western Individual.
Journal of medical virologyEBV and post-transplant lymphoproliferative disorder: a complex relationship.
Hematology. American Society of Hematology. Education ProgramFrom the archives of MD Anderson Cancer Center: EBV-positive fibrin-associated large B-cell lymphoma in an ovarian leiomyoma with cystic degeneration: A case report and discussion of differential diagnosis.
Annals of diagnostic pathologyDecoding Critical Targets and Signaling Pathways in EBV-Mediated Diseases Using Large Language Models.
VirusesHodgkin lymphoma: the role of EBV plasma viral load testing in an HIV-endemic setting.
Clinical and experimental medicineDistinct epigenetic and transcriptional profiles of Epstein-Barr virus-positive and negative primary CNS lymphomas.
Neuro-oncologyEpstein-Barr virus-associated B-cell lymphoproliferative disorders and lymphomas: Diagnostic overlaps and defining features.
Human pathologyPosttransplant Lymphoproliferative Disorder in Pediatric Solid-Organ Transplant Recipients: A 7-Year Single-Center Analysis.
Experimental and clinical transplantation : official journal of the Middle East Society for Organ TransplantationUbiquitin-Mediated Effects on Oncogenesis during EBV and KSHV Infection.
VirusesGenetic and immunological features of immune deficiency and dysregulation-associated lymphoproliferations and lymphomas as a basis for classification.
HistopathologyLymphoproliferative Disorder in an Esophageal Cancer Patient Treated with Pembrolizumab.
Internal medicine (Tokyo, Japan)The role of c-Myc in Epstein-Barr virus-associated cancers: Mechanistic insights and therapeutic implications.
Microbial pathogenesisB Cell Differentiation and the Origin and Pathogenesis of Human B Cell Lymphomas.
Methods in molecular biology (Clifton, N.J.)Hemophagocytic lymphohistiocytosis in a patient with Epstein-Barr virus-positive diffuse large B-cell lymphoma treated with chimeric antigen receptor T-cell therapy.
ImmunotherapyNodal T-Cell Lymphoma Transdifferentiated from Mantle Cell Lymphoma with Epstein-Barr Virus Infection.
Pathobiology : journal of immunopathology, molecular and cellular biologyDetection of anti-EBV TCR CDR3s associated with better outcomes for EBV-positive, Ugandan cases of Burkitt lymphoma.
International journal of cancerFibrin associated large B-cell lymphoma accidentally identified in a breast implant capsule: a molecular report of a rare entity.
PathologicaBreast Implant-Related EBV + Diffuse Large B-Cell Lymphoma-Case Report and Review of the Literature.
International journal of surgical pathologyCirculating Tumor DNA Sequencing for Biologic Classification and Individualized Risk Stratification in Patients With Hodgkin Lymphoma.
Journal of clinical oncology : official journal of the American Society of Clinical OncologyEpstein-Barr virus-positive plasmacytoma in an immunocompetent female: A case report.
Journal of clinical and experimental hematopathology : JCEHEpstein-Barr virus-related lymphoproliferative disorders of the skin.
Dermatology reportsPost-transplant lymphoproliferative disorders after allogeneic hematopoietic stem cell transplantation: a case report, meta-analysis, and systematic review.
Diagnostic pathologyThe DNA loop release factor WAPL suppresses Epstein-Barr virus latent membrane protein expression to maintain the highly restricted latency I program.
PLoS pathogensAdult T-cell leukemia/lymphoma with angioimmunoblastic T-cell lymphoma-like feature and molecularly confirmed RHOA Gly 17 Val (G17V) mutation: A case report.
Pathology internationalOral manifestations of peripheral T cell lymphoma, not otherwise specified: case series and review of the current literature.
Oral surgery, oral medicine, oral pathology and oral radiologyXMEN-associated Systemic EBV-positive T-cell Lymphoma of Childhood: Report of Two Cases and Literature Review.
Journal of pediatric hematology/oncologySystemic EBV-positive T-cell lymphoma of childhood with unusual cytogenetic findings and family history of Still's disease-associated recurrent macrophage activation syndrome.
Pediatric blood & cancerStem cell memory EBV-specific T cells control EBV tumor growth and persist in vivo.
Science advancesResistance mechanisms and potential therapeutic strategies in relapsed or refractory natural killer/T cell lymphoma.
Chinese medical journalClinicopathological and Molecular Characteristics of Rare EBV-associated Diffuse Large B-cell Lymphoma With IRF4 Rearrangement.
The American journal of surgical pathologyMechanistic Understanding of EBV+Lymphoproliferative Disease Development After Transplantation.
TransplantationClinicopathologic Insights and Molecular Oncogenesis: Understanding Epstein-Barr Virus-Induced B-cell Lymphoproliferations.
International journal of surgical pathologyLMP1 enhances aerobic glycolysis in natural killer/T cell lymphoma.
Cell death & diseaseEnhancing diagnostic precision in EBV-related HLH: a multifaceted approach using 18F-FDG PET/CT and nomogram integration.
Cancer imaging : the official publication of the International Cancer Imaging SocietyEpstein-Barr Virus-Associated Smooth Muscle Tumor After Kidney Transplantation: A French Multicenter Retrospective Study.
Clinical transplantationHIV infection is associated with compromised tumor microenvironment adaptive immune reactivity in Hodgkin lymphoma.
Blood advancesEnhanced prognostic evaluation of diffuse large B-cell lymphoma: A comprehensive surveillance study incorporating Epstein-Barr virus infection status and immunohistochemical markers.
Journal of medical virologyCombined Flow-Fluorescence in situ hybridization to HHV-8 and EBV reveals the viral heterogeneity of primary effusion lymphoma.
Journal of medical virologyEpstein-Barr virus-driven lymphoproliferation in inborn errors of immunity: a diagnostic and therapeutic challenge.
Expert review of clinical immunologyImpact of prophylaxis with rituximab on EBV-related complications after allogeneic hematopoietic cell transplantation in children.
Frontiers in immunologyA proteome-wide analysis unveils a core Epstein-Barr virus antibody signature of classic Hodgkin lymphoma across ethnically diverse populations.
International journal of cancerPost-transplant lymphoproliferative disorder risk and outcomes in renal transplant patients treated with belatacept immunosuppression.
Frontiers in transplantationAssociações
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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.
- Molecular pathogenesis and evolving treatment landscape of extranodal NK/T-cell lymphoma.Proceedings of the Japan Academy. Series B, Physical and biological sciences· 2026· PMID 41813156mais citado
- Epstein-Barr virus infection in children with liver transplantation.
- Classic hodgkin lymphoma associated with Epstein-Barr virus reactivation during vedolizumab therapy for ulcerative colitis.
- A multidimensional workflow profiling of allogeneic virus-specific T cell therapies reveals potency-linked signatures.
- Withaferin A inhibits EBV-driven lymphomagenesis through multiple mechanisms, including EBNA1 degradation.
- [Clinical Characteristics and Prognostic Analysis of Patients with EBV-Positive Diffuse Large B-Cell Lymphoma].
- [EBNA1 Knockdown Modulates Proliferation, Apoptosis, and Metabolism in Burkitt's Lymphoma Cells by Downregulating miR-127-5p].
- A 43-Year-Old Man With Isolated Cutaneous Lymphomatoid Granulomatosis Presenting as a Chronic Necrotizing Ulcer of the Upper Arm.
- Composite lymphoma composed of follicular lymphoma and nodal T-follicular helper cell lymphoma: report of 3 cases highlighting histopathologic zonation of each neoplastic component.
- Temporal association of diffuse large B-cell lymphoma with PD-1 inhibitor therapy in a patient with gastric adenocarcinoma: a case report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:289644(Orphanet)
- MONDO:0017343(MONDO)
- GARD:21147(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q55786997(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
