title: A boy with fatal infectious mononucleosis suspected as the first Japanese case of X-linked lymphoproliferative syndrome.
abstract: We report a case of a 10-month-old boy who died of severe hepatic failure after a prolonged course of infectious mononucleosis. He also presented interstitial pneumonitis, meningoencephalitis and aplastic anaemia. Epstein-Barr virus (EBV)-specific cytotoxic T lymphocyte (CTL) activity had not been detected in his peripheral blood during the course of the illness. Studies of his mother revealed a severe reactivation pattern of anti-EBV antibodies and decreased EBV-specific CTL activity. An X-linked familial susceptibility to EBV infection such as X-linked lymphoproliferative syndrome (XLP) might be associated with his fatal EBV infection.
authors.affiliation: Department of Pediatrics, Sapporo Medical University School of Medicine, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Tsutsumi
authors.forename: H
authors.initials: H
authors.lastname: Kamazaki
authors.forename: M
authors.initials: M
authors.lastname: Satoh
authors.forename: S
authors.initials: S
authors.lastname: Imai
authors.forename: S
authors.initials: S
authors.lastname: Nakata
authors.forename: S
authors.initials: S
authors.lastname: Chiba
mesh_terms: D003602:Cytotoxicity, Immunologic; D008040:Genetic Linkage; D006501:Hepatic Encephalopathy / Q000209:etiology; D004854:Herpesvirus 4, Human / Q000276:immunology; D006801:Humans; D007223:Infant; D007244:Infectious Mononucleosis / Q000150:complications*; D008232:Lymphoproliferative Disorders / Q000150:complications* / Q000235:genetics*; D008297:Male; D014960:X Chromosome