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<p>Single-system, single-site (SS-s) Langerhans cell histiocytosis (LCH) is usually managed with observation or local therapy; however, a subset of patients requires systemic chemotherapy, although the optimal intensity for this group remains unclear. In this study, we retrospectively reviewed patients with newly diagnosed or recurrent SS-s LCH who were treated at our institution. They received 6&#xa0;weeks of induction chemotherapy with cytarabine, vincristine, and prednisolone, followed by 1&#xa0;year of oral maintenance therapy with 6-mercaptopurine and methotrexate. Of these patients, 15 were evaluable (13 newly diagnosed and 2 recurrent). At the end of induction, all patients achieved a good or partial response and were administered maintenance therapy, after which they achieved a good response. With a median follow-up of 5.6&#xa0;years, all patients were alive without subsequent relapse, death, or central nervous system (CNS) complications. No grade 3 or 4 nonhematologic toxicities were observed, except transient increases in liver enzymes during maintenance therapy. No adverse events occurred that required hospitalization. These results suggest that cytarabine-based induction followed by oral maintenance may be an effective and tolerable option for patients with SS-s LCH requiring systemic therapy.</p>

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Cytarabine-based induction and oral maintenance therapy for a single system with single-site Langerhans cell histiocytosis

  • 期刊论文

  • 发表日期：

    2026年7月3日

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  • Yuichi Mitani,
  • Kohei Fukuoka,
  • Mamoru Honda,
  • Koichi Oshima,
  • Makiko Mori,
  • Yutaka Tanami,
  • Noriko Watanabe,
  • Azusa Watanabe,
  • Jun Kurihara,
  • Katsuaki Taira,
  • Atsuko Nakazawa,
  • Yuki Arakawa,
  • Katsuyoshi Koh

摘要

Single-system, single-site (SS-s) Langerhans cell histiocytosis (LCH) is usually managed with observation or local therapy; however, a subset of patients requires systemic chemotherapy, although the optimal intensity for this group remains unclear. In this study, we retrospectively reviewed patients with newly diagnosed or recurrent SS-s LCH who were treated at our institution. They received 6 weeks of induction chemotherapy with cytarabine, vincristine, and prednisolone, followed by 1 year of oral maintenance therapy with 6-mercaptopurine and methotrexate. Of these patients, 15 were evaluable (13 newly diagnosed and 2 recurrent). At the end of induction, all patients achieved a good or partial response and were administered maintenance therapy, after which they achieved a good response. With a median follow-up of 5.6 years, all patients were alive without subsequent relapse, death, or central nervous system (CNS) complications. No grade 3 or 4 nonhematologic toxicities were observed, except transient increases in liver enzymes during maintenance therapy. No adverse events occurred that required hospitalization. These results suggest that cytarabine-based induction followed by oral maintenance may be an effective and tolerable option for patients with SS-s LCH requiring systemic therapy.