title: Immunophenotypic analysis of Epstein-Barr virus (EBV)-infected CD8(+) T cells in a patient with EBV-associated hemophagocytic lymphohistiocytosis.
abstract: Hemophagocytic lymphohistiocytosis (HLH) is a severe and often fatal condition characterized by uncontrolled activation of T cells and macrophages. In Epstein-Barr virus (EBV)-associated HLH (EBV-HLH), the pathogenic roles of ectopic EBV infection in the T-cell population and of clonal proliferation of EBV-infected T cells has been described. However, the immunophenotype of EBV-infected T cells has not been fully characterized. Here we describe a case of EBV-HLH presenting with a massive clonal proliferation of CD8(+) T cells with TCR VB14. Analysis of in situ hybridization for EBV-encoded small RNA1 showed that only CD8(+) T cells harbored EBV in this patient. The EBV-infected TCR VB14(+) CD8(+) T cells exhibited unique immunophenotypic features including lacked CD5 expression and a markedly bright expression of HLA-DR. After initiation of treatment with prednisolone, etoposide, and cyclosporin A, the percentage of infected cells declined progressively in parallel with other serum markers such as ferritin. These findings suggest that lacking expression of CD5 on CD8(+) T cells with specific TCR VB may serve as a useful marker of dysregulated T-cell activation and proliferation in EBV-HLH.
authors.affiliation: Department of Pediatrics, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan. taizo@ped.m.kanazawa-u.ac.jp
authors.forename: Taizo
authors.initials: T
authors.lastname: Wada
authors.forename: Toshiro
authors.initials: T
authors.lastname: Kurokawa
authors.forename: Tomoko
authors.initials: T
authors.lastname: Toma
authors.forename: Fumie
authors.initials: F
authors.lastname: Shibata
authors.forename: Yumi
authors.initials: Y
authors.lastname: Tone
authors.forename: Yoko
authors.initials: Y
authors.lastname: Hashida
authors.forename: Hiroyasu
authors.initials: H
authors.lastname: Kaya
authors.forename: Takashi
authors.initials: T
authors.lastname: Yoshida
authors.forename: Akihiro
authors.initials: A
authors.lastname: Yachie
mesh_terms: D000328:Adult; D018414:CD8-Positive T-Lymphocytes / Q000821:virology*; D005260:Female; D004854:Herpesvirus 4, Human / Q000302:isolation & purification*; D006801:Humans; D016130:Immunophenotyping; D051359:Lymphohistiocytosis, Hemophagocytic / Q000276:immunology* / Q000821:virology