ArticleJournal of clinical immunology2015
Late-onset severe chronic active EBV in a patient for five years with mutations in STXBP2 (MUNC18-2) and PRF1 (perforin 1).
Article in Journal of clinical immunology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
17 citing papers in PubMed, 42 citations in OpenAlex.
- Epstein-Barr virus-associated B-cell lymphoproliferative disorder meeting the definition of CAEBV B cell disease: a case report.BMC infectious diseases · 2023Article
- Review
- T Cell-Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis (HLH) Occurs in Non-Asians and Is Associated with a T Cell Activation State that Is Comparable to Primary HLH.Journal of clinical immunology · 2021Article
- Review
- Inherited Genetic Susceptibility to Nonimmunosuppressed Epstein-Barr Virus-associated T/NK-cell Lymphoproliferative Diseases in Chinese Patients.Current medical science · 2021Article
- Germline Compound Heterozygous Variants Identified in theFrontiers in pediatrics · 2021Article
- Immune Escape by Non-coding RNAs of the Epstein Barr Virus.Frontiers in microbiology · 2021Review
- Vaccination against the Epstein-Barr virus.Cellular and molecular life sciences : CMLS · 2020Review
- Chronic Active Epstein-Barr Virus Infection: Is It Immunodeficiency, Malignancy, or Both?Cancers · 2020Review
- Immunodeficiencies that predispose to pathologies by human oncogenic γ-herpesviruses.FEMS microbiology reviews · 2019Review
- Epstein-Barr Virus-Associated γδ T-Cell Lymphoproliferative Disorder Associated With HypomorphicFrontiers in pediatrics · 2019Article
- Clinical and genetic analysis of immunodeficiency-related diseases associated withPediatric investigation · 2018Article
- Recurrent scarring papulovesicular lesions on sun-exposed skin in a 22-year-old man.Journal of the American Academy of Dermatology · 2018Article
- MPEG1/perforin-2 mutations in human pulmonary nontuberculous mycobacterial infections.JCI insight · 2017Article
- Chronic Active Epstein-Barr Virus Disease.Frontiers in immunology · 2017Review
- Epstein-Barr Virus-Specific Immune Control by Innate Lymphocytes.Frontiers in immunology · 2017Review
- 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
7 authors at 5 institutions in 1 country.
Funding
Abstract
Severe chronic active Epstein-Barr virus (CAEBV) disease is defined as a severe progressive illness lasting 6 months or longer with infiltration of tissues with EBV-positive lymphocytes, markedly elevated levels of EBV DNA in the blood, and no known immunodeficiency such as HIV. These patients usually have fever, splenomegaly, lymphadenopathy, and may have markedly elevated EBV antibody titers to viral capsid antigen. Although the cause of most cases of severe CAEBV is unknown, one well-documented case was associated with compound heterozygous mutations in PRF1 (perforin 1). Here we report a patient with prolonged severe CAEBV who underwent bone marrow transplant for his disease and subsequently was found to have compound heterozygous mutations in STXBP2 (MUNC18-2) as well as a heterozygous mutation in PRF1 (perforin 1).
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.