Chronic Active Epstein-Barr Virus Infection: Is It Immunodeficiency, Malignancy, or Both?
EBV
EBV-positive T/NK-cell lymphoproliferative disease
Epstein–Barr virus
chronic active EBV infection
hydroa vacciniforme
immunodeficiency
lymphoma
severe mosquito bite allergy
Journal
Cancers
ISSN: 2072-6694
Titre abrégé: Cancers (Basel)
Pays: Switzerland
ID NLM: 101526829
Informations de publication
Date de publication:
30 Oct 2020
30 Oct 2020
Historique:
received:
18
09
2020
revised:
17
10
2020
accepted:
27
10
2020
entrez:
4
11
2020
pubmed:
5
11
2020
medline:
5
11
2020
Statut:
epublish
Résumé
Chronic active Epstein-Barr virus (EBV) infection (CAEBV) is a rare syndrome characterized by prolonged infectious mononucleosis-like symptoms and elevated peripheral blood EBV DNA load in apparently immunocompetent persons. CAEBV has been primarily reported in East Asia and Latin America, suggesting a genetic predisposition in its pathogenesis. In most cases of CAEBV, EBV induces proliferation of its unusual host cells, T or natural killer (NK) cells. The clinical course of CAEBV is heterogeneous; some patients show an indolent course, remaining in a stable condition for years, whereas others show an aggressive course with a fatal outcome due to hemophagocytic lymphohistiocytosis, multiple organ failure, or progression to leukemia/lymphoma. The pathogenesis of CAEBV is unclear and clinicopathological investigations suggest that it has aspects of both malignant neoplasm and immunodeficiency. Recent genetic analyses of both viral and host genomes in CAEBV patients have led to discoveries that are improving our understanding of the nature of this syndrome. This article summarizes the latest findings on CAEBV and discusses critical unsolved questions regarding its pathogenesis and disease concept.
Identifiants
pubmed: 33143184
pii: cancers12113202
doi: 10.3390/cancers12113202
pmc: PMC7692233
pii:
doi:
Types de publication
Journal Article
Review
Langues
eng
Subventions
Organisme : Japan Agency for Medical Research and Development
ID : JP17ek0109098
Organisme : National Center for Child Health and Development
ID : 30-21
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