郎格汉斯组织细胞增多症的消化系表现、诊断及治疗

Digestive system manifestations, diagnosis and treatment of Langerhans cell histiocytosis

郎格汉斯组织细胞增多症是一种少见的全身多系统受侵犯的组织细胞异常增生性疾病,常见受累部位为骨、皮肤、肺脏、骨髓、淋巴结等,除此之外,尚可侵犯肝脏、胆道及胃肠道等消化器官,临床表现与其他消化系统疾病相比缺乏特异性,故诊断难度较大.一旦郎格汉斯组织细胞增多症患者明确诊断消化系统受累,则需要系统性治疗,甚至是肝脏移植.本文综述郎格汉斯组织细胞增多症的消化系统表现,从而对其早期诊断及治疗提供帮助.

Langerhans cell histiocytosis （LCH） is a rare disease characterized by an abnormal proliferation of histiocytes, known as Langerhans cells （LCs）. At present, the pathogenesis of LCH remains unknown. LCH often involves the bone, skin, lung, bone marrow and lymph nodes. Besides, the liver, bile duct and gastrointestinal tract may also be affected. LCH has no specifi c clinical manifestations compared to other digestive system diseases. Once digestive system involvement is diagnosed in LCH patients, prompt treatment （even liver transplantation） should be given. In this paper, we will review the digestive system manifestations, diagnosis and treatment of LCH.