ArticleBlood advances2022
High risk of relapsed disease in patients with NK/T-cell chronic active Epstein-Barr virus disease outside of Asia.
Article in Blood advances, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 27 citations in OpenAlex.
- Time-Dependent Prognostic Value of the sCD25-to-Ferritin Ratio in Chronic Active Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis.Infectious diseases and therapy · 2026Article
- Fatal refractory chronic active Epstein-Barr virus infection with hemophagocytic lymphohistiocytosis and NK/T-cell lymphoma: a case report.ASM case reports · 2026Article
- Avoidance of related donors in CAEBV with germline immune variants: long-termoutcome of matched unrelated donor HSCT - a case report.Annals of hematology · 2026Article
- Molecular mechanisms of Epstein-Barr Virus in the pathogenesis of lymphomas and new opportunities for precision medicine.Discover oncology · 2026Review
- Cardiovascular complications in chronic active Epstein-Barr virus infection: a narrative review.Frontiers in cardiovascular medicine · 2026Review
- EBV-Driven HLH and T Cell Lymphoma in a Child with X-Linked Agammaglobulinemia: A Genetically Confirmed Case Report and Literature Review.Journal of personalized medicine · 2025Article
- Case Report: Hydroa vacciniforme-like lymphoproliferative disorder, an EBV-associated disease, successfully treated with hematopoietic stem cell transplantation.Frontiers in immunology · 2025Article
- Hepatic tuberculosis induced by rituximab treatment for C1q nephropathy with minimal change disease: a case report.Frontiers in medicine · 2025Article
- A differential diagnosis method for systemic CAEBV and the prospect of EBV-related immune cell markers via flow cytometry.Annals of medicine · 2024Review
- Role of rapidly evolving immunotherapy in chronic active Epstein-Barr virus disease.Frontiers in immunology · 2024Review
- EBV-Encoded MicroRNA-BART17-3p Targets DDX3X and Promotes EBV Infection in EBV-Associated T/Natural Killer-Cell Lymphoproliferative Diseases.Open forum infectious diseases · 2023Article
- Updated guidelines for chronic active Epstein-Barr virus disease.International journal of hematology · 2023Review
- Chronic active Epstein-Barr virus and hydroa vacciniforme-like lymphoproliferative disorder in a pediatric patient complicated by fatal ruptured cerebral artery aneurysm.Clinical case reports · 2023Article
- Review
- Epstein-Barr Virus Genome Deletions in Epstein-Barr Virus-Positive T/NK Cell Lymphoproliferative Diseases.Journal of virology · 2022Article
- Allogeneic hematopoietic stem cell transplantation for the treatment of chronic active Epstein-Barr virus infection.Cell transplantationReview
Corrections and comments
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Authors and funding
29 authors at 20 institutions in 4 countries.
Funding
Abstract
Chronic active Epstein-Barr virus (EBV) disease (CAEBV) is characterized by high levels of EBV predominantly in T and/or natural killer cells with lymphoproliferation, organ failure due to infiltration of tissues with virus-infected cells, hemophagocytic lymphohistiocytosis, and/or lymphoma. The disease is more common in Asia than in the United States and Europe. Although allogeneic hematopoietic stem cell transplantation (HSCT) is considered the only curative therapy for CAEBV, its efficacy and the best treatment modality to reduce disease severity prior to HSCT is unknown. Here, we retrospectively assessed an international cohort of 57 patients outside of Asia. Treatment of the disease varied widely, although most patients ultimately proceeded to HSCT. Though patients undergoing HSCT had better survival than those who did not (55% vs 25%, P < .01), there was still a high rate of death in both groups. Mortality was largely not affected by age, ethnicity, cell-type involvement, or disease complications, but development of lymphoma showed a trend with increased mortality (56% vs 35%, P = .1). The overwhelming majority (75%) of patients who died after HSCT succumbed to relapsed disease. CAEBV remains challenging to treat when advanced disease is present. Outcomes would likely improve with better disease control strategies, earlier referral for HSCT, and close follow-up after HSCT including aggressive management of rising EBV DNA levels in the blood.
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