A boy with fatal infectious mononucleosis suspected as the first Japanese case of X-linked lymphoproliferative syndrome.
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.
Tsutsumi
H
H
Department of Pediatrics, Sapporo Medical University School of Medicine, Japan.
Kamazaki
H
H
Satoh
M
M
Imai
S
S
Nakata
S
S
Chiba
S
S
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