成人朗格汉斯细胞组织细胞增生症临床特征以及预后因素分析  

Clinical Features and Potential Prognostic Factors of Langerhans Cell Histiocytosis in Adult

作　　者：凌彦弢 李亚荣 龚玉萍[1] Ling Yantao;Li Yarong;Gong Yuping(Department of Hematology,West China Hospital,Sichuan University,Chengdu,Sichuan 610041,China)

机构地区：[1]四川大学华西医院血液内科,四川成都610041

出　　处：《四川医学》2021年第2期137-141,共5页Sichuan Medical Journal

摘　　要：目的分析成人朗格汉斯细胞组织细胞增生症(LCH)的临床特征和潜在的预后因素。方法回顾性纳入我院血液科2007年至2017年确诊的53例成人LCH患者,收集临床基本资料、疗效及预后数据,分析患者不同临床特点对疾病疗效及预后的影响。结果 53例成人LCH患者中男女性别比为2.8∶1,发病年龄中位数为34岁。单系统单病灶LCH(SS-LCH)缓解中位时间为83个月,单系统多病灶LCH(SM-LCH)、多系统LCH(MS-LCH)缓解中位时间分别为36个月、40个月,明显低于SS-LCH(P=0.004)。相关性分析提示LCH受累范围与LDH值呈正相关(P=0.026)。多因素分析提示,与SS-LCH患者相比,MS-LCH和SM-LCH疾病复发风险更高(P=0.047);而初始选择联合治疗方案(手术联合放疗)显著增加LCH复发风险(P=0.022)。在排除年龄因素下,复发(P=0.047)和血清LDH高水平(P=0.023)是LCH治疗效果不佳的危险因素。结论 LCH以男性为主,起始治疗采用联合方案以及疾病累及范围广是LCH复发的独立危险因素。复发患者和高血清LDH水平是LCH治疗效果不佳的主要影响因素。Objective To analyze the clinical features and potential prognostic factors of adult Langerhans cell histiocytosis(LCH).Methods We retrospectively included 53 adult patients with LCH diagnosed from the department of hematology,west China hospital,Sichuan university from 2007 to 2017.Basic clinical data,efficacy and prognostic data were collected,and the impact of different clinical characteristics of patients on efficacy and prognosis was analyzed.Results The sex ratio of 53 adult LCH patients was 2. 8 ∶ 1,in which median age of was 34 years. The median remission time for single-system LCH(SS-LCH) was 83 months,and the median remission time for single-system multifocal LCH(SM-LCH) and multi-system LCH(MS-LCH) were 36 months and 40 months,respectively,who were significantly lower than SS-LCH(P = 0. 004).Correlation analysis suggested that the involvement of LCH was positively correlated with the LDH value(P = 0. 026).Multivariate analysis suggested that compared with SS-LCH,MS-LCH and SM-LCH had a higher risk of recurrence(P = 0. 047);while the initial combination therapy option significantly increased the risk of LCH recurrence(P = 0. 022).When excluding age,disease recurrence(P = 0. 047) and high serum LDH levels(P = 0. 023) were risk factors for poor efficacy of LCH.Conclusion Adult LCH is predominantly male.The combination therapy in initial treatment and a wide involvement of LCHare an independent risk factor for LCH recurrence.Disease recurrence and high serum LDH levels can reduce the efficacy of LCH.

关 键 词：朗格汉斯细胞组织细胞增生症 临床特征 预后因素 

分 类 号：R551.1[医药卫生—血液循环系统疾病]