title: Severe Epstein-Barr virus infection in primary immunodeficiency and the normal host.
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.
authors.affiliation: Department of Immunology, Great Ormond Street Hospital, London, UK.|Molecular and Cellular Immunology Section, UCL Institute of Child Health, London, UK.
authors.forename: Austen J J
authors.initials: AJ
authors.lastname: Worth
authors.affiliation: Infection, Inflammation and Rheumatology Section, UCL Institute of Child Health, London, UK.
authors.forename: Charlotte J
authors.initials: CJ
authors.lastname: Houldcroft
authors.affiliation: Department of Immunology, Great Ormond Street Hospital, London, UK.|Molecular and Cellular Immunology Section, UCL Institute of Child Health, London, UK.
authors.forename: Claire
authors.initials: C
authors.lastname: Booth
mesh_terms: D015551:Autoimmunity; D002472:Cell Transformation, Viral; D002908:Chronic Disease; D004198:Disease Susceptibility; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000175:diagnosis / Q000821:virology*; D020022:Genetic Predisposition to Disease; D014644:Genetic Variation; D016679:Genome, Viral; D004854:Herpesvirus 4, Human / Q000502:physiology*; D054884:Host-Pathogen Interactions / Q000235:genetics / Q000276:immunology; D006801:Humans; D007109:Immunity; D016867:Immunocompromised Host; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000175:diagnosis / Q000209:etiology / Q000378:metabolism; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications / Q000175:diagnosis / Q000209:etiology; D008232:Lymphoproliferative Disorders / Q000209:etiology; D009369:Neoplasms / Q000209:etiology; D012720:Severity of Illness Index; D014775:Virus Activation
keywords: 
  Epstein-Barr virus
  ; haemophagocytosis; primary immunodeficiency