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.

abstractsObjectives 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.