Pathogenesis of chronic active Epstein-Barr virus infection: Is this an infectious disease, lymphoproliferative disorder, or immunodeficiency?
Version of Record online: 21 JUN 2006
Copyright © 2006 John Wiley & Sons, Ltd.
Reviews in Medical Virology
Volume 16, Issue 4, pages 251–261, July/August 2006
How to Cite
Kimura, H. (2006), Pathogenesis of chronic active Epstein-Barr virus infection: Is this an infectious disease, lymphoproliferative disorder, or immunodeficiency?. Rev. Med. Virol., 16: 251–261. doi: 10.1002/rmv.505
- Issue online: 5 JUL 2006
- Version of Record online: 21 JUN 2006
- Manuscript Accepted: 13 MAR 2006
- Manuscript Revised: 2 MAR 2006
- Manuscript Received: 23 JAN 2006
Chronic active Epstein-Barr virus infection (CAEBV) is characterised by chronic or recurrent infectious mononucleosis-like symptoms, such as fever, hepatosplenomegaly, persistent hepatitis and extensive lymphadenopathy. Patients with CAEBV have high viral loads in their peripheral blood and/or an unusual pattern of EBV-related antibodies. This disease is rare but severe with high morbidity and mortality. Nearly three decades have passed since this disease was first identified, and recent advances in technology have increased our understanding of CAEBV pathophysiology. There is accumulating evidence that the clonal expansion of EBV-infected T or natural killer (NK) cells plays a central role in the pathogenesis of CAEBV. However, it remains unclear whether CAEBV is truly a monoclonal lymphoproliferative disorder. EBV-infected T or NK cells are able to evade the host cellular immune system due to the limited expression of viral proteins of reduced antigenicity. Recent studies suggest that infection of T or NK cells is a common event during primary EBV infection. A defect or single nucleotide polymorphism in host immune-modulating genes may allow for the expansion of virus infected cells giving rise to CAEBV. In this review, I summarise our current understanding of the pathogenesis of CAEBV and propose a model of CAEBV pathogenicity. Copyright © 2006 John Wiley & Sons, Ltd.