Management of severe neutropenia with cyclosporin during initial treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis

Shinsaku Imashuku, Shigeyoshi Hibi, Kikuko Kuriyama, Yasuhiro Tabata, Tetsuo Hashida, Asayuki Iwai, Masahiko Kato, Nobuko Yamashita, Megumi Oda, Masashi Uchida, Naoko Kinugawa, Machiko Sawada, Mutsuko Konno

Research output: Contribution to journalArticle

32 Citations (Scopus)

Abstract

Severe neutropenia (absolute neutrophil count <500/μl) 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.

Original languageEnglish
Pages (from-to)339-346
Number of pages8
JournalLeukemia and Lymphoma
Volume36
Issue number3-4
Publication statusPublished - 2000

Fingerprint

Hemophagocytic Lymphohistiocytosis
Neutropenia
Human Herpesvirus 4
Cyclosporine
Neutrophils
Etoposide
Adrenal Cortex Hormones
Therapeutics
Infection

Keywords

  • Cyclosporin A
  • Epstein-Barr virus
  • Hemophagocytic lymphohistiocytosis
  • Neutropenia

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

Cite this

Imashuku, S., Hibi, S., Kuriyama, K., Tabata, Y., Hashida, T., Iwai, A., ... Konno, M. (2000). Management of severe neutropenia with cyclosporin during initial treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis. Leukemia and Lymphoma, 36(3-4), 339-346.

Management of severe neutropenia with cyclosporin during initial treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis. / Imashuku, Shinsaku; Hibi, Shigeyoshi; Kuriyama, Kikuko; Tabata, Yasuhiro; Hashida, Tetsuo; Iwai, Asayuki; Kato, Masahiko; Yamashita, Nobuko; Oda, Megumi; Uchida, Masashi; Kinugawa, Naoko; Sawada, Machiko; Konno, Mutsuko.

In: Leukemia and Lymphoma, Vol. 36, No. 3-4, 2000, p. 339-346.

Research output: Contribution to journalArticle

Imashuku, S, Hibi, S, Kuriyama, K, Tabata, Y, Hashida, T, Iwai, A, Kato, M, Yamashita, N, Oda, M, Uchida, M, Kinugawa, N, Sawada, M & Konno, M 2000, 'Management of severe neutropenia with cyclosporin during initial treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis', Leukemia and Lymphoma, vol. 36, no. 3-4, pp. 339-346.
Imashuku, Shinsaku ; Hibi, Shigeyoshi ; Kuriyama, Kikuko ; Tabata, Yasuhiro ; Hashida, Tetsuo ; Iwai, Asayuki ; Kato, Masahiko ; Yamashita, Nobuko ; Oda, Megumi ; Uchida, Masashi ; Kinugawa, Naoko ; Sawada, Machiko ; Konno, Mutsuko. / Management of severe neutropenia with cyclosporin during initial treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis. In: Leukemia and Lymphoma. 2000 ; Vol. 36, No. 3-4. pp. 339-346.
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