title: Clonal expansion of Epstein-Barr virus (EBV)-infected γδ T cells in patients with chronic active EBV disease and hydroa vacciniforme-like eruptions.
abstract: Chronic active Epstein-Barr virus (EBV) disease (CAEBV) is a systemic EBV-positive lymphoproliferative disorder characterized by fever, lymphadenopathy, and splenomegaly. Patients with CAEBV may present with cutaneous symptoms, including hypersensitivity to mosquito bites and hydroa vacciniforme (HV)-like eruptions. HV is a rare photodermatosis characterized by vesicles and crust formation after exposure to sunlight, with onset in childhood, and is associated with latent EBV infection. While γδ T cells have recently been demonstrated to be the major EBV-infected cell population in HV, the immunophenotypic features of EBV-infected γδ T cells in CAEBV with HV-like eruptions or HV remain largely undetermined. We describe three patients with CAEBV whose γδ T cells were found to be the major cellular target of EBV. HV-like eruptions were observed in two of these patients. A clonally expanded subpopulation of γδ T cells that were highly activated and T cell receptor Vγ9- and Vδ2-positive cells was demonstrated in all patients. We also show that the clonally expanded γδ T cells infiltrated into the HV-like eruptions in one patient from whom skin biopsy specimens were available. These results suggest the pathogenic roles of clonally expanded γδ T cells infected by EBV in patients with CAEBV and HV-like eruptions.
authors.affiliation: Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, 13-1 Takaramachi, Kanazawa 920-8641, Japan. taizo@staff.kanazawa-u.ac.jp
authors.forename: Taizo
authors.initials: T
authors.lastname: Wada
authors.forename: Akiko
authors.initials: A
authors.lastname: Toga
authors.forename: Yasuhisa
authors.initials: Y
authors.lastname: Sakakibara
authors.forename: Tomoko
authors.initials: T
authors.lastname: Toma
authors.forename: Minoru
authors.initials: M
authors.lastname: Hasegawa
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Takehara
authors.forename: Tomonari
authors.initials: T
authors.lastname: Shigemura
authors.forename: Kazunaga
authors.initials: K
authors.lastname: Agematsu
authors.forename: Akihiro
authors.initials: A
authors.lastname: Yachie
mesh_terms: D000293:Adolescent; D044466:Asian People; D002648:Child; D020031:Epstein-Barr Virus Infections / Q000235:genetics / Q000276:immunology* / Q000378:metabolism; D005260:Female; D006801:Humans; D006837:Hydroa Vacciniforme / Q000276:immunology*; D016130:Immunophenotyping; D007564:Japan; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000235:genetics* / Q000378:metabolism; D013601:T-Lymphocytes / Q000276:immunology* / Q000378:metabolism / Q000821:virology
chemical_list: D016692:Receptors, Antigen, T-Cell, gamma-delta