title: Development of Langerhans cell histiocytosis associated with chronic active Epstein-Barr virus infection.
abstract: Chronic active Epstein-Barr virus (CAEBV) infection is characterized by a status of lymphoproliferative disease of EBV-infected cells, resulting in chronic or recurrent infectious mononucleosis-like symptoms. CAEBV is always accompanied by life-threatening complications. We report the case of a 2-year-old female patient with CAEBV who subsequently developed Langerhans cell histiocytosis (LCH) presenting with bilateral exophthalmos, bone, and skin involvement. In situ hybridization for EBER revealed EBV-infected B-cells present in lesional tissue implying that interactions between EBV-infected B-cells and lesional Langerhans cells may be associated with the development of LCH.
authors.affiliation: Department of Pediatrics, Kinki University School of Medicine, Osaka, Japan. nsakata@med.kindai.ac.jp
authors.forename: Naoki
authors.initials: N
authors.lastname: Sakata
authors.forename: Naomi
authors.initials: N
authors.lastname: Toguchi
authors.forename: Masatomo
authors.initials: M
authors.lastname: Kimura
authors.forename: Masahiro
authors.initials: M
authors.lastname: Nakayama
authors.forename: Keisei
authors.initials: K
authors.lastname: Kawa
authors.forename: Tsukasa
authors.initials: T
authors.lastname: Takemura
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001402:B-Lymphocytes / Q000821:virology; D002675:Child, Preschool; D002908:Chronic Disease; D016572:Cyclosporine / Q000627:therapeutic use; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000821:virology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017403:In Situ Hybridization; D007223:Infant; D009916:Orbital Diseases / Q000188:drug therapy / Q000473:pathology / Q000821:virology; D016133:Polymerase Chain Reaction; D011239:Prednisolone / Q000627:therapeutic use; D012367:RNA, Viral / Q000032:analysis; D012871:Skin Diseases / Q000188:drug therapy / Q000473:pathology / Q000821:virology; D013601:T-Lymphocytes / Q000821:virology
chemical_list: D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D012367:RNA, Viral; D016572:Cyclosporine; D011239:Prednisolone