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Severe Epstein-Barr virus infection in primary immunodeficiency and the normal host

Worth, AJJ; Houldcroft, CJ; Booth, C; (2016) Severe Epstein-Barr virus infection in primary immunodeficiency and the normal host. British Journal Of Haematology , 175 (4) pp. 559-576. 10.1111/bjh.14339. Green open access

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Abstract

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.

Type: Article
Title: Severe Epstein-Barr virus infection in primary immunodeficiency and the normal host
Open access status: An open access version is available from UCL Discovery
DOI: 10.1111/bjh.14339
Publisher version: http://dx.doi.org/10.1111/bjh.14339
Language: English
Additional information: Copyright © 2016 John Wiley & Sons Ltd. This is the peer reviewed version of the following article: [Worth, A. J. J., Houldcroft, C. J. and Booth, C. (2016), Severe Epstein–Barr virus infection in primary immunodeficiency and the normal host. British Journal of Haematology, 175: 559–576. doi: 10.1111/bjh.14339], which has been published in final form at DOI: 10.1111/bjh.14339. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Self-Archiving.
Keywords: Epstein-Barr virus, primary immunodeficiency, haemophagocytosis
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Infection, Immunity and Inflammation Dept
URI: https://discovery.ucl.ac.uk/id/eprint/1527022
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