朗格汉斯细胞组织细胞增生症3例并文献复习

Clinical analysis of 3 patients with Langerhans' cell histiocytosis

目的探讨郎格汉斯细胞组织细胞增生症(LCH)的临床特征、诊断和治疗。方法回顾性分析3例LCH患者的诊疗过程,并进行相关文献复习。结果 3例患者均为成年发病,2例患者以肝功能异常为主要表现,其中1例伴有肺、甲状腺和皮肤受累,另1例伴有颅骨受累;1例患者以骨质破坏为主要表现。3例患者均经过病理学诊断为LCH。结论 LCH是一种综合征,症状随受累器官多少和部位而不同。临床上出现不明原因的皮疹、肝脾肿大、反复肝功能异常、突眼、尿崩、颅骨缺损和骨质破坏等表现时,应考虑LCH可能。治疗选择及预后与受累器官数目和功能受损情况有密切联系。

Objective To explore the clinical features,diagnosis and treatment of Langerhans＇ cell histiocytosis（LCH）.Methods The clinical data including the process of diagnosis and treatment of 3 cases of LCH were retrospectively analyzed in addition with review of literatures.Results All these 3 cases were adult,two of them mainly manifested as hepatic dysfunction and in one of these 2 cases also involved lung,thyroid and skin,the other one involved skull and the remaining one case mainly manifested as bone destruction.They were diagnosed as LCH by pathological examination.Conclusion LCH is a kind of clinical syndrome,and its symptoms may be variable according to the involvement of organs or parts of the body.Awareness of the diagnosis of LCH is crucial,when patients have skin rashes with unknown origin,splenohepatomegalia,repeated hepatic dysfunction,exophthalmos,diabetes insipidus,skull defect and destruction of bone etc,the diagnosis of LCH should be under consideration.The choice for its treatment and its prognosis may be based on the extent of involvement of organs and impairment of function.