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Severe Epstein–Barr virus infection in primary immunodeficiency and the normal host
Author(s) -
Worth Austen J. J.,
Houldcroft Charlotte J.,
Booth Claire
Publication year - 2016
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.14339
Subject(s) - asymptomatic , immunology , primary immunodeficiency , epstein–barr virus infection , immunodeficiency , disease , virus , malignancy , medicine , epstein–barr virus , chronic infection , viral disease , lymphoproliferative disorders , common variable immunodeficiency , virology , biology , lymphoma , immune system , pathology , antibody
Summary Epstein–Barr virus ( EBV ) infection is ubiquitous in humans, but the majority of infections have an asymptomatic or self‐limiting clinical course. Rarely, individuals may develop a pathological EBV infection with a variety of life threatening complications (including haemophagocytosis and malignancy) and others develop asymptomatic chronic EBV viraemia. Although an impaired ability to control EBV infection has long been recognised as a hallmark of severe T‐cell immunodeficiency, the advent of next generation sequencing has identified a series of Primary Immunodeficiencies in which EBV ‐related pathology is the dominant feature. Chronic active EBV infection is defined as chronic EBV viraemia associated with systemic lymphoproliferative disease, in the absence of immunodeficiency. Descriptions of larger cohorts of patients with chronic active EBV in recent years have significantly advanced our understanding of this clinical syndrome. In this review we summarise the current understanding of the pathophysiology and natural history of these diseases and clinical syndromes, and discuss approaches to the investigation and treatment of severe or atypical EBV infection.