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Severe Epstein-Barr virus infection in primary immunodeficiency and the normal host
Austen J J Worth1,2, Charlotte J Houldcroft3, Claire Booth1,2
1Department of Immunology, Great Ormond Street Hospital, London, UK.
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.
Insights
Epstein-Barr virus (EBV) infection is usually mild, but severe cases can cause life-threatening complications. This review explores chronic active EBV infection and severe EBV disease, advancing understanding and treatment strategies.
Area of Science:
- Virology
- Immunology
- Hematology
Background:
- Epstein-Barr virus (EBV) infection is widespread, typically asymptomatic or self-limiting.
- Rarely, EBV causes severe complications like hemophagocytosis, malignancy, or chronic viremia.
- Primary immunodeficiencies increasingly recognized for dominant EBV-related pathology.
Purpose of the Study:
- To review the pathophysiology and natural history of severe and atypical EBV infections.
- To discuss diagnostic and therapeutic approaches for EBV-related diseases.
- To synthesize current knowledge on chronic active EBV infection.
Main Methods:
- Literature review of recent cohort descriptions and clinical studies.
- Synthesis of data on EBV pathophysiology and natural history.
- Discussion of diagnostic criteria and treatment strategies.
Main Results:
- Significant advancements in understanding chronic active EBV infection (CAEBV) and EBV-associated lymphoproliferative disorders.
- Identification of primary immunodeficiencies with EBV pathology as a key feature.
- Improved insights into the spectrum of severe EBV complications.
Conclusions:
- Severe EBV infections and chronic active EBV infection represent distinct clinical entities requiring specific management.
- Further research is needed to elucidate the mechanisms underlying EBV pathogenesis in susceptible individuals.
- Optimized investigation and treatment protocols are crucial for improving patient outcomes.
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