title: Current research on chronic active Epstein-Barr virus infection in Japan.
abstract: Epstein-Barr virus (EBV) infection is usually asymptomatic and persists lifelong. Although EBV-infected B cells have the potential for unlimited proliferation, they are effectively removed by the virus-specific cytotoxic T cells, and EBV-associated lymphoproliferative disease develops only in immunocompromised hosts. Rarely, however, individuals without apparent immunodeficiency develop chronic EBV infection with persistent infectious mononucleosis-like symptoms. These patients have high EBV-DNA load in the peripheral blood and systemic clonal expansion of EBV-infected T cells or natural killer (NK) cells. Their prognosis is poor with life-threatening complications including hemophagocytic lymphohistiocytosis, organ failure, and malignant lymphomas. The term "chronic active EBV infection" (CAEBV) is now generally used for this disease. The geographical distribution of CAEBV is markedly uneven and most cases have been reported from Japan and other East Asian countries. Here we summarize the current understanding of CAEBV and describe the recent progress of CAEBV research in Japan.
authors: Department of Infectious Diseases, National Research Institute for Child Health and Development, Tokyo, Japan.
authors: Shigeyoshi
authors: S
authors: Fujiwara
authors: Hiroshi
authors: H
authors: Kimura
authors: Ken-ichi
authors: K
authors: Imadome
authors: Ayako
authors: A
authors: Arai
authors: Eiichi
authors: E
authors: Kodama
authors: Tomohiro
authors: T
authors: Morio
authors: Norio
authors: N
authors: Shimizu
authors: Hiroshi
authors: H
authors: Wakiguchi
mesh_terms: D000818:Animals; D035843:Biomedical Research; D002648:Child; D002908:Chronic Disease; D004195:Disease Models, Animal; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D006801:Humans; D007564:Japan; D051379:Mice
keywords: EBV-associated T/NK-cell lymphoproliferative disease; EBV-associated hemophagocytic lymphohistiocytosis; Epstein-Barr virus; chronic active EBV infection; flow-cytometric in situ hybridization; hydroa vacciniforme; hypersensitivity to mosquito bites; mouse model