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A Syndrome of Peripheral Blood T-Cell Infection With Epstein-Barr
Virus (EBV) Followed by EBV-Positive T-Cell Lymphoma
Hirokazu Kanegane,
Kishor Bhatia,
Marina Gutierrez,
Hisashi Kaneda,
Taizo Wada,
Akihiro Yachie,
Hidetoshi Seki,
Takashi Arai,
Sei-ichi Kagimoto,
Minoru Okazaki,
Tsutomu Oh-ishi,
Amir Moghaddam,
Fred Wang, and
Giovanna Tosato
From the Center for Biologics Evaluation and Research, Bethesda MD;
National Cancer Institute, National Institutes of Health, Bethesda,
Maryland; Kanazawa University School of Medicine, Kanazawa, Ishikawa,
Japan; Saitama Children's Medical Center, Iwatsuki, Saitama, Japan;
and the Infectious Disease Division, Brigham and Women's Hospital,
Harvard Medical School, Boston, MA.
The role of Epstein-Barr virus (EBV) in the pathogenesis of severe,
chronic active EBV infection and its complications is unclear. We
investigated two Japanese patients diagnosed with severe, chronic
active EBV infection who subsequently developed EBV-positive T-cell
lymphoma. The patients displayed abnormally high antibody titers to EBV
antigens, and had evidence of peripheral blood CD4+
T-cell infection with EBV, 19 months and 3 months, respectively, before
the diagnosis of EBV-positive T-cell lymphoma. The lymphomas were
infected with monoclonal EBV and expressed the EBV latency genes
EBNA-1, LMP-1, and LMP-2. Genetic studies showed that the virus
detected in the T-cell lymphoma was indistinguishable, with respect to
type and previously defined LMP-1 and EBNA-1 gene variations, from
virus detected in the peripheral blood T cells 19 months earlier. These
studies support an important pathogenetic role of T-cell infection with
EBV in chronic active EBV infection and in the EBV-positive T-cell
lymphoma that followed.
Blood, Vol. 91 No. 6 (March 15), 1998:
pp. 2085-2091
© 1998 by The American Society of Hematology.
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