title: Rituximab and cyclosporine therapy for accelerated phase Chediak-Higashi syndrome.
abstract: A 19-month-old male with Chediak-Higashi syndrome developed Epstein-Barr virus (EBV)-associated accelerated phase. Real-time polymerase chain reaction showed high EBV-DNA levels in plasma and peripheral blood mononuclear cells. His condition was refractory to conventional treatments for hemophagocytic lymphohistiocytosis, including corticosteroids, cyclosporine, and etoposide. In situ hybridization revealed higher proportion of EBER-1-positive cells in CD19+ cell fraction than in CD8+ cell fraction. Complete remission was achieved by combination therapy with rituximab and cyclosporine; subsequent bone marrow transplantation was successful. Combination therapy with rituximab and cyclosporine could be effective in patients with EBV-infected T and B cells.
authors.affiliation: Division of Infectious Disease, Immunology, and Allergy, Saitama Children's Medical Center, Saitama, Japan. chikara.ohgimi.510311@gmail.com
authors.forename: Chikara
authors.initials: C
authors.lastname: Ogimi
authors.forename: Risa
authors.initials: R
authors.lastname: Tanaka
authors.forename: Takashi
authors.initials: T
authors.lastname: Arai
authors.forename: Akira
authors.initials: A
authors.lastname: Kikuchi
authors.forename: Ryoji
authors.initials: R
authors.lastname: Hanada
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Oh-Ishi
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D002609:Chediak-Higashi Syndrome / Q000188:drug therapy* / Q000503:physiopathology / Q000821:virology; D016572:Cyclosporine / Q000627:therapeutic use*; D004279:DNA, Viral / Q000032:analysis; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D017403:In Situ Hybridization; D007223:Infant; D008297:Male; D020133:Reverse Transcriptase Polymerase Chain Reaction; D000069283:Rituximab
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D004279:DNA, Viral; D007155:Immunologic Factors; D000069283:Rituximab; D016572:Cyclosporine