成人朗格汉斯细胞组织细胞增多症11例临床特征及EB病毒感染分析  

Clinical analysis of 11 cases of adult Langerhans cell histiocytosis and Epstein-Barr infection

作　　者：肖萍萍[1] 王行富[2] 曾志勇[1] 陈君敏[1] XIAO Ping-ping;WANG Xing-fu;ZENG Zhi-yong;CHEN Jun-min(Department of Hematology,First Affiliated Hospital of Fujian Medical Universily,Fuzhou 350005,China)

机构地区：[1]福建医科大学附属第一医院血液科,福建福州350005 [2]福建医科大学附属第一医院病理科,福建福州350005

出　　处：《中国实用内科杂志》2020年第7期555-557,585,共4页Chinese Journal of Practical Internal Medicine

基　　金：国家自然科学基金(编号81400160和81272628);福建省自然科学基金(编号2017J01185);吴阶平医学基金(编号320.6750.19094-41和320.6750.19094-58)。

摘　　要：目的分析成人朗格汉斯细胞组织细胞增多症(langerhans cell histiocytosis,LCH)患者临床特点,比较EB病毒(Epstein-Barr virus,EBV)阳性组与阴性组临床差异。方法收集2013年2月至2018年2月福建医科大学附属第一医院血液科收治的11例成人LCH病例资料,包括临床表现、实验室检查、影像学和治疗;荧光定量PCR检测患者血清中EBV DNA拷贝数。结果 11例LCH成人患者中,男性7例,女性4例,中位年龄为38岁(22~58岁),临床表现以骨痛最常见,其次是发热和淋巴结肿大;其中10例接受手术治疗,11例随访结束全部存活;6例确诊为EBV感染,EBV阳性组β2-微球蛋白水平高于EBV阴性组(P<0.05);EBV阳性组Ki-67增殖指数明显升高(P<0.05);EBV阳性组肿块较EBV阴性组体积更大(P<0.05)。结论 EBV感染在成人LCH在中较常见,可能影响LCH的Ki-67增殖指数和病灶大小。Objective To explore the clinical features,treatment and prognosis of adult Langerhans cell histiocytosis(LCH)and to compare the clinical characteristic differences between EBV-positive and EBV-negative groups. Methods We included 11 adult LCH patients, who were admitted to Department of Hematology, the First Affiliated Hospital of Fujian Medical University from February 2013 to February 2018.Data on clinical manifestations, laboratory tests, imaging, and treatments were collected. Real-time Polymerase Chain Reaction(PCR) was used to detect genome copies of EBV DNA in serum. Results Among 11 adult LCH patients, 7 were male and 4 were female. The median age was 38 years(22-58 years). The most common clinical feature was bone pain,followed by fever and lymphadenectasis. Ten patients received surgery,and the 11 patients survived at the end of follow-up. Six of them were diagnosed with EBV infection. The level of serum β2-microglobulin in EBV-positive group was higher than that of EBV-negative group(P<0.05).Ki-67 proliferation index was significantly increased in EBV positive group(P<0.05). The size of lesion in EBV-positive group was larger than that of EBV-negative group(P<0.05). Conclusion EBV infection is common in adult LCH, which may influence Ki-67 proliferation index and lesion size in adult LCH.

关 键 词：朗格汉斯细胞组织细胞增多症 成人 EB病毒 

分 类 号：R597[医药卫生—内科学]