Severe chronic active Epstein-Barr virus infection syndrome

M. Okano, S. Matsumoto, T. Osato, Y. Sakiyama, G. M. Thiele, D. T. Purtilo

Research output: Contribution to journalArticlepeer-review

149 Scopus citations


Reports of unusually severe lymphoproliferative disorders associated with extremely high antibody titers against Epstein-Barr virus (EBV) have recently increased. The syndrome, which we designated severe chronic active EBV infection syndrome, is characterized by persistent or intermittent fever, lymphadenopathy, and hepatosplenomegaly and primarily affects children and young adults. Polyclonal gammopathy and bone marrow suppression are generally observed, and some patients develop B-cell or T-cell lymphoproliferation or lymphoma. Frequently, EBV genomes are detectable in tissues infiltrated with lymphoid cells. Additionally, it is difficult to establish spontaneous or B95-8 EBV-induced cell lines despite the expression of an activated EBV infection. We review and report here the published medical literature and our own experience regarding patients with severe chronic active EBV infection syndrome in an attempt to understand this enigmatic syndrome and the possible pathogenetic mechanism(s) responsible for this disorder.

Original languageEnglish (US)
Pages (from-to)129-135
Number of pages7
JournalClinical Microbiology Reviews
Issue number1
StatePublished - Jan 1991

ASJC Scopus subject areas

  • General Medicine


Dive into the research topics of 'Severe chronic active Epstein-Barr virus infection syndrome'. Together they form a unique fingerprint.

Cite this