Abstract:

Objective To analyze the clinical efficacy，timing of transplantation，prognosis and comprehensive management strategies of liver transplantation for langerhans cell histiocytosis（LCH）in children. Methods A case of liver transplantation for langerhans cell histiocytosis in children was analyzed. Databases of CNKI，Wanfang，VIP，PubMed and Web of Science（as of August 31，2025）were retrieved to collect the case reports on liver transplantation for langerhans cell histiocytosis（LCH）in children，and the main clinical data of the patients were analyzed descriptively. Results In Shanghai Children’s Hospital，a male patient with multisystem involvement LCH received chemotherapy and liver transplantation，experiencing no complications and achieving complete remission for over six years. A comprehensive literature review encompassed 15 studies， involving 39 patients in total. The male-to-female ratio was 0.95：1. The median age at LCH diagnosis was 22（6～48）months，and the median age at liver transplantation was 50（14～204）months. All patients exhibited liver involvement，with the skin being the most commonly affected site（71 .8%）. Thirty-two patients had involvementof three or more organs. Pathological confirmation of LCH was obtained for all cases，with skin biopsy representing the most frequent diagnostic method（59 .0%）. The median follow-up duration was 20 months（9 d～88 months）. Among the 39 patients，recurrence was observed in 7 cases，death occurred in 4 cases，vascular complications were reported in 2 cases，and the most prevalent infection was cytomegalovirus infection（33 .3%）. Conclusion Liver transplantation is an effective treatment for end-stage liver disease related to Langerhans cell histiocytosis in children. To ensure sustained complete remission，this approach should be integrated with chemotherapy，targeted therapy， and meticulous follow-up protocols.

Key words:

text-indent:0pt, vertical-align:middle, "> Liver transplantation, Langerhans cell histiocytosis, Children

摘要：

目的 探讨肝移植治疗儿童朗格汉斯组织细胞增生症（langerhans cell histiocytosis，LCH）的临床疗效、时机、预后及综合管理策略。 方法 分析1 例肝移植治疗儿童LCH 的临床病例，检索中国知网、万方、维普、PubMed、Web of Science 数据库（截至2025 年8 月31 日），收集肝移植治疗儿童LCH 的病例，对患者主要临床资料进行描述性分析。 结果 上海市儿童医院1 例男性多系统受累LCH 患者，经化疗及肝移植，无并发症，疾病完全缓解6 年多。检索数据库共纳入文献15 篇，涉及患者39 例，男女比例为0.95：1。确诊LCH 中位年龄为22（6～48）个月，肝移植中位年龄为50（14～204）个月。所有患者均有肝脏受累，另外皮肤是最常见受累部位（71 .8%），32 例患者受累器官数≥3 个。所有患者均经病理诊断为LCH，皮肤活检病理最多见（59 .0%）。中位随访时间为20 个月（9 d～88 个月），39 例患者中复发7 例，死亡4 例，血管并发症2 例，胆道并发症1 例，最常见的感染为巨细胞病毒感染（33 .3%）。 结论 肝移植是儿童朗格汉斯组织细胞增多症相关终末期肝病的有效治疗手段，需整合化疗、靶向治疗和密切随访以实现持续完全缓解。

关键词:

text-indent:0pt, vertical-align:middle, "> 肝移植, 朗格汉斯组织细胞增生症, 儿童