朗格汉斯细胞组织细胞增生症肝损伤的相关研究及进展

Research and Advances on Langerhans Cell Histiocytosis-Related Liver Injury

摘要: 朗格汉斯细胞组织细胞增生症(Langerhans Cell Histiocytosis, LCH)是一种以病理性朗格汉斯细胞克隆性增殖为特征的罕见疾病，主要影响免疫系统，近年来研究发现其与多脏器损伤之间存在潜在关联，尤其是肝损伤。肝脏作为重要的代谢和解毒器官，其损伤可能导致严重的临床后果。尽管已有一些关于LCH的研究探讨其发病机制和临床表现，但现阶段对LCH导致肝损伤的具体病理机制仍不够清晰，且临床管理策略的制定亦缺乏系统性指导。本综述旨在综合近年来的研究成果，探讨LCH与肝损伤之间的关系，分析其潜在的病理机制、临床表现以及目前的治疗策略，以期为临床实践提供参考和指导，推动对该疾病的进一步研究与理解。

Abstract: Langerhans Cell Histiocytosis (LCH) is a rare disease characterized by the pathological clonal proliferation of Langerhans cells, primarily affecting the immune system. Recent studies have found a potential association between LCH and multi-organ damage, particularly liver injury. The liver, as an important metabolic and detoxification organ, can lead to severe clinical consequences when damaged. Although some studies have explored the pathogenesis and clinical manifestations of LCH, the specific pathological mechanisms by which LCH causes liver injury remain unclear, and there is a lack of systematic guidance for clinical management strategies. This review aims to synthesize recent research findings, explore the relationship between LCH and liver injury, analyze its potential pathological mechanisms, clinical manifestations, and current treatment strategies, in order to provide reference and guidance for clinical practice and promote further research and understanding of this disease.