title: Fatal Epstein-Barr virus-associated hemophagocytic syndrome.
abstract: A virus-associated hemophagocytic syndrome is characterized by high fever, liver dysfunction, coagulation abnormalities, pancytopenia, and a benign histiocytic proliferation with prominent hemophagocytosis in bone marrow, lymph node, spleen, and liver. We describe six Japanese children with fatal Epstein-Barr virus (EBV)-associated hemophagocytic syndrome. Five of the six patients had serologic evidence of primary EBV infection at the onset of their diseases. EBV genomes were detected in all the patients by Southern blot hybridization or the polymerase chain reaction. Furthermore, clonality analysis of the EBV genome showed that EBV-infected cells proliferated monoclonally or biclonally in three examined patients. In situ hybridization study using EBV-encoded RNA 1 (EBER1) showed that EBER1 was detected in one of two examined liver tissues, which localized in hepatocytes.
authors.affiliation: Department of Pediatrics, Hokkaido University School of Medicine, Sapporo, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Kikuta
authors.forename: Y
authors.initials: Y
authors.lastname: Sakiyama
authors.forename: S
authors.initials: S
authors.lastname: Matsumoto
authors.forename: T
authors.initials: T
authors.lastname: Oh-Ishi
authors.forename: T
authors.initials: T
authors.lastname: Nakano
authors.forename: T
authors.initials: T
authors.lastname: Nagashima
authors.forename: T
authors.initials: T
authors.lastname: Oka
authors.forename: T
authors.initials: T
authors.lastname: Hironaka
authors.forename: K
authors.initials: K
authors.lastname: Hirai
mesh_terms: D001483:Base Sequence; D002648:Child; D002675:Child, Preschool; D004279:DNA, Viral / Q000032:analysis; D005260:Female; D006566:Herpesviridae Infections / Q000382:microbiology* / Q000401:mortality; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000472:pathogenicity*; D015616:Histiocytosis, Non-Langerhans-Cell / Q000382:microbiology* / Q000401:mortality; D006801:Humans; D007223:Infant; D008297:Male; D008969:Molecular Sequence Data; D016133:Polymerase Chain Reaction; D012367:RNA, Viral / Q000032:analysis; D014412:Tumor Virus Infections
chemical_list: D004279:DNA, Viral; D012367:RNA, Viral