title: Success with infliximab in treating refractory hemophagocytic lymphohistiocytosis.
abstract: Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder characterized by fever, pancytopenia, hepatosplenomegaly, liver dysfunction, and hemophagocytosis. A 29-year-old woman, diagnosed with systemic lupus erythematosus in 1996, developed HLH in early June 2002. HLH remained refractory during 1.5 months of treatment including corticosteroid, cyclosporine, plasma exchange, vincristine, and etoposide. Infliximab (5 mg/kg/day) was then administered twice. After the second administration, the patient attained remission. Because HLH itself is not a neoplasm but an uncontrolled immune reaction, blocking cytokines involved in the reaction should have therapeutic potentials. For HLH patients not responding to conventional therapy, anticytokine treatment with infliximab may represent one of promising options.
authors.affiliation: Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, Fukuoka, Japan.
authors.forename: Tomoko
authors.initials: T
authors.lastname: Henzan
authors.forename: Koji
authors.initials: K
authors.lastname: Nagafuji
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Tsukamoto
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Miyamoto
authors.forename: Hisashi
authors.initials: H
authors.lastname: Gondo
authors.forename: Shinsaku
authors.initials: S
authors.lastname: Imashuku
authors.forename: Mine
authors.initials: M
authors.lastname: Harada
mesh_terms: D000328:Adult; D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D018501:Antirheumatic Agents / Q000008:administration & dosage*; D005260:Female; D006801:Humans; D000069285:Infliximab; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology; D010951:Plasma Exchange; D012074:Remission Induction
chemical_list: D000911:Antibodies, Monoclonal; D018501:Antirheumatic Agents; D000069285:Infliximab