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目的:探讨朗汉斯细胞组织细胞增生症(LCH)的临床表现、诊断分型、治疗与随访。方法:回顾性分析LCH的临床资料。结果:103例符合LCH可信度的三级诊断标准。其中<2岁13例,2～<5岁28例,5～<15岁28例,≥15岁34例;男64例,女39例。单系统LCH(SS-LCH)79例,多系统LCH(MS-LCH)24例。LCH以骨受累最常见。SS-LCH以单纯手术治疗为主;MS-LCH以联合化疗为主。随访48例,治愈和好转37例,复发6例,死亡5例,1例出现二次肿瘤;2例出现后遗症,失访55例。结论:①SS-LCH较MS-LCH多见,骨骼是最常见的受损器官;②放射影像学检查对早期发现LCH病变有意义,骨损害时更易在中轴骨的发现病变;③SS-LCH多采用单纯手术治疗,MS-LCH以联合化疗为主;④SS-LCH疗效和预后较MS-LCH好。
Objective: To investigate the clinical manifestations, diagnosis, classification, treatment and follow-up of Langerhans cell histiocytosis (LCH). Methods: The clinical data of LCH were retrospectively analyzed. Results: Three hundred three patients met the three criteria of LCH credibility. Among them, <2 years in 13 cases, 2 ~ <5 years in 28 cases, 5 ~ <15 years in 28 cases, ≥ 15 years in 34 cases; 64 males and 39 females. 79 single-system LCH (SS-LCH) and 24 multi-system LCH (MS-LCH). LCH is the most common bone involvement. SS-LCH mainly surgery alone; MS-LCH combined chemotherapy. A total of 48 cases were followed up, 37 cases were cured and improved, 6 cases relapsed, 5 cases died, 1 case had secondary tumor, 2 cases had sequelae and 55 cases were lost. Conclusion: ①SS-LCH is more common than MS-LCH, skeletal is the most common organ damage; ② Radiographic examination is meaningful for early detection of LCH lesions, more susceptible to the detection of axial bone lesions; ③SS-LCH Simple surgical treatment, MS-LCH combined chemotherapy; ④SS-LCH efficacy and prognosis than MS-LCH good.