title: Management of severe neutropenia with cyclosporin during initial treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis.
abstract: Severe neutropenia (absolute neutrophil count <500/gl) is probably due to the combined effects of dysregulated cytokine production and chemotherapeutic agents, and is one of the risk factors in the initial treatment of patients with Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH). We report here 9 cases of neutropenic HLH, of which 8 were treated with cyclosporin (CSA, 2-6 mg/kg/day; continuous infusion, or 6 mg/kg/day; per os, for periods ranging from 9 days to >8 weeks) in the initial neutropenic phase during induction treatment using corticosteroids and etoposide. Five of the 6 cases, in which CSA treatment was started early (before the second week of induction), survived the critical period with recovery of neutrophil counts within a week. The remaining 3 cases, in which CSA was introduced later or not at all, died of infection. Based on these results, we recommend a prompt short-term CSA infusion during neutropenic episodes in the most common treatment regimen of etoposide and corticosteroids in patients with HLH. Improved neutrophil recovery as a result of CSA treatment makes it possible to continue immunochemotherapy safely and obtain improved patient outcomes.
authors.affiliation: Division of Pediatrics, Children's Research Hospital, Kyoto Prefectural University of Medicine Japan. shinim95@mbox.kyoto-inet.or.jp
authors.forename: S
authors.initials: S
authors.lastname: Imashuku
authors.forename: S
authors.initials: S
authors.lastname: Hibi
authors.forename: K
authors.initials: K
authors.lastname: Kuriyama
authors.forename: Y
authors.initials: Y
authors.lastname: Tabata
authors.forename: T
authors.initials: T
authors.lastname: Hashida
authors.forename: A
authors.initials: A
authors.lastname: Iwai
authors.forename: M
authors.initials: M
authors.lastname: Kato
authors.forename: N
authors.initials: N
authors.lastname: Yamashita
authors.forename: M
authors.initials: M
authors.lastname: Oda MUchida
authors.forename: N
authors.initials: N
authors.lastname: Kinugawa
authors.forename: M
authors.initials: M
authors.lastname: Sawada
authors.forename: M
authors.initials: M
authors.lastname: Konno
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D016572:Cyclosporine / Q000627:therapeutic use*; D005260:Female; D004854:Herpesvirus 4, Human; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications / Q000188:drug therapy* / Q000821:virology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D008262:Macrophage Activation / Q000187:drug effects; D008297:Male; D009503:Neutropenia / Q000188:drug therapy* / Q000209:etiology
chemical_list: D007166:Immunosuppressive Agents; D016572:Cyclosporine