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

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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
DOIs
Publication statusPublished - Jan 1 2000

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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., 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, 36(3-4), 339-346. https://doi.org/10.3109/10428190009148855