Abstract
Clinical findings and immunological abnormalities in patients with chronic active Epstein-Barr (EB) virus infection (CAEBV) were reviewed. The most common symptoms of this disease consist of prolonged fever and lymphoproliferative syndrome. EB-virus specific and nonspecific cellular immunities have been reported to be defective, and these defective immune functions might be corresponding to the development of CAEBV. T/ NK cells are mainly infected with EB-virus, and clonal proliferation of these cell lineages was frequently observed in CAEBV. Several immune abnormalities were also observed in parents of the patients, and NK cell activity of the patients and their mothers were well correlated. Curiously, clinical signs and immunological abnormalities similar to CAEBV were observed in some patients with autoimmune lymphoproliferative syndrome (ALPS). Although CAEBV is a critical disease with poor prognosis, diagnostic criterion and the methods for treatment have not been established. Bone marrow transplantation and adoptive immunotherapy with EB-virus specific cytotoxic T lymphocytes are considered effective in some patients.
