Langerhans细胞组织细胞增生症258例临床病理特征和免疫表型分析  

Langerhans cell histiocytosis： a clinicopathologic and immunohistochemical analysis of 258 cases

作　　者：许霞[1] 刘卫平[1] 杨群培[1] 王威亚[1] 廖殿英[1] 赵莎[1] 毕成峰[1] 林莉[1] 闵敏[1] 

机构地区：[1]四川大学华西医院病理科,成都610041

出　　处：《中华病理学杂志》2012年第2期91-96,共6页Chinese Journal of Pathology

基　　金：国家自然科学基金（30971113）

摘　　要：目的 观察Langerhans细胞组织细胞增生症（LCH）的临床病理特征,了解langerin、CD1a及S-100蛋白表达在LCH诊断中的作用.方法 对1992年1月至2008年12月18年间四川大学华西医院病理科档案资料诊断的258例LCH进行了临床和组织病理学的回顾性研究.采用免疫组织化学EnVision法,检测CD1 a、langerin及S-100蛋白的表达情况.结果 258例LCH患者年龄≥16岁或≤2岁的分别为126例（48.8％）和37例（14.3％）,其余95例（36.8％）患者的年龄介于二者之间.临床上,258例LCH共有364处病变,其中骨骼病变281处（77.2％）,且以颅骨累及为多,有112处（39.9％）,其次为淋巴结25处（6.9％）和皮肤14处（3.8％）.201例（77.9％）为单系统、单病灶；21例（8.1％）为单系统、多个病灶；26例（10.1％）为多系统、多个病灶；2例（0.8％）为肺LCH,8例（3.1％）不能分类.组织学上,258例LCH共265个样本中均见Langerhans细胞增生,166例（62.6％）见不等量多核巨细胞；嗜酸性粒细胞是主要的非肿瘤性细胞成分,57例（21.5％）可见嗜酸性脓肿；29例（10.9％）有片状凝固性坏死；124例（46.8％）可见死骨.免疫表型检测,100％（206/206）病例之瘤细胞表达CD1a,S-100蛋白和langerin的表达率分别为99.1％ （209/211）和98.5％（193/196）,三者的阳性表达率差异无统计学意义（P＞0.05）.结论 此组LCH患者以成年患者为多,多系统病变所占比例较低.在LCH中,langerin、CDla及S-100蛋白表达的敏感性差异无统计学意义,CDla及langerin是LCH诊断必不可少的标志物.Objectives To observe the clinicopathologic features of Langerhans cell histiocytosis （LCH）,and to evaluate the values of langerin,CD1a and S-100 protein expression in diagnosis of the tumor.Methods Total 258 cases of Langerhans cell histiocytosis in the past 18 years （ from 1992 to 2008 ）were collected,morphologic review and immunohistochemical staining were performed. Results In all 258 cases,the ages of patients older than 16 years or younger than 2 years were 126 （48.8%） and 37 （ 14.3% ）,respectively,in the remaining 95 （36.8% ） of the cases,the age of the patients ranged from 2 to 16 years.For all of 258 cases,there were 364 diseased sites. Bony lesions accounted for 77.2%（281 cases）,especially the skull （112 cases,39.9% ）,followed by lymph node （25 cases,6.9% ） and skin （ 14 cases,3.8% ）.Clinically,unisystem or unifocal disease was predominant （201 cases,77.9% ）,followed by unisystem and muhifocal disease （21 cases, 8.1%）, multi-system disease （26 cases,10.1% ）,isolated pulmonary LCH （2 cases,0.8% ）,and unclassified （8 cases,3.1% ）.Histologically,variable number of Langerhans cells was present in 265 samples of 258 cases.Muhinucleated giant cells were found in 166 （62.6% ） of the samples.Eosinophils were the major infiltrating non-neoplastic cells,and eosinophilic abscess was seen in 57 cases （21.5%）.Coagulative necrosis and dead bone were detected in 29 （ 10.9% ） and 124 （46.8% ） of the cases,respectively.Immunohistochemically,the expression of S-100 protein,CD1 a and langerin was 99.1% （209/211）,100% （206/206） and 98.5% （193/196）,respectively,and the sensitivity of them had no statistical difference.Conclusions In this group of LCH cases,the ratio of adult patients is high,but the proportion of multi-organ lesion is low. No significant difference of the sensitivity is found among langerin,CD1a and S-100 expression in diagnosis of LCH.

关 键 词：组织细胞增多症 郎格尔汉斯细胞 免疫表型分型 

分 类 号：R55[医药卫生—血液循环系统疾病]