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Severe chronic active Epstein-Barr virus infection syndrome
M Okano1, S Matsumoto, T Osato
1Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha 68198-3135.
Clinical Microbiology Reviews
|January 1, 1991
Summary
Severe chronic active Epstein-Barr virus (EBV) infection syndrome presents with severe lymphoproliferative disorders and high EBV antibody titers, primarily affecting young individuals. This review explores its characteristics and potential pathogenetic mechanisms.
Area of Science:
- Virology
- Immunology
- Oncology
Background:
- Increasing reports link severe lymphoproliferative disorders to high Epstein-Barr virus (EBV) antibody titers.
- A distinct syndrome, severe chronic active EBV infection syndrome, has been identified, predominantly affecting children and young adults.
Purpose of the Study:
- To review and report on severe chronic active EBV infection syndrome.
- To understand the clinical manifestations and potential pathogenetic mechanisms of this enigmatic disorder.
Main Methods:
- Review of published medical literature.
- Analysis of clinical experience with patients diagnosed with severe chronic active EBV infection syndrome.
Main Results:
- The syndrome is characterized by persistent fever, lymphadenopathy, hepatosplenomegaly, polyclonal gammopathy, and bone marrow suppression.
- EBV genomes are often detected in affected tissues, and patients may develop B-cell or T-cell lymphoproliferation or lymphoma.
- Establishing EBV-induced cell lines can be challenging despite active infection.
Conclusions:
- Severe chronic active EBV infection syndrome represents a significant clinical entity with complex pathogenesis.
- Further research is needed to elucidate the mechanisms driving this severe EBV-associated disorder.