Langerin在朗格汉斯细胞组织细胞增生症中的表达及诊断价值

Expression of Langerin in and its diagnostic value for Langerhans cell histiocytosis

目的 Langerin是近年来新发现的朗格汉斯细胞标志物,能诱导朗格汉斯细胞内Birbeck颗粒的形成,对朗格汉斯细胞具有高度选择性。文中探讨Langerin在朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)的组织细胞中的表达及诊断价值。方法收集23例LCH和18例其他非LCH的组织细胞疾病(non-Langerhans cell histiocytic disorder,N-LCHD)的患者资料,进行病理组织学观察,免疫组织化学染色(EnVision法)检测Langerin、CD1a和S-100蛋白的表达。结果组织学显示23例LCH都有病理性朗格汉斯组织细胞增生,细胞呈卵圆形,较大,核不规则、有核沟和核折叠,核仁不明显,核膜薄,细胞质中等丰富,略呈嗜酸性,伴多量嗜酸性粒细胞浸润。免疫组织化学染色示LCH和N-LCHD中Langerin的表达率分别为100%和0%(P<0.05),CD1a的表达率分别为95.65%和5.6%(P<0.05),S-100蛋白的表达率分别为100%和72.2%。Langerin、CD1a和S-100蛋白对诊断LCH的敏感性分别为100%、95.65%和100%,特异性分别为100%、94.4%和27.8%。结论 Langerin是对诊断LCH高度敏感且特异的标志物,有助于LCH的诊断和鉴别诊断。

Objective Langerin is a newly discovered specific immunomarker of Langerhans cells, which plays a critical role in the formation of Birbeck granules. The aim of this study was to assess the specificity and utility of Langerin in the diagnosis of Langerhans cell histiocytosis （LCH）. Methods We retrieved the data of 23 cases of LCH and 18 cases of various non- Langerhans cell histiocytic disorders （N-LCHD） from pathology files, and determined the expressions of Langerin, CDla and S-100 protein of both LCH and N-LCHD by pathohistological observation and immunohistochemical examination. Results Histologically, all the 23 cases of LCH showed proliferation of large oval ceils with indented, grooved or folded nuclei, inconspicuous nucleoli, thin nuclear membranes, and moderately abundant and slightly eosinophilic cytoplasm, accompanied by infiltration of variable numbers of eosinophils. The expressions of Langerin in LCH and N-LCHD were 100% and 0%, respectively （P 〈0.05）, those of CDla were 95.65% and 5.6% （P 〈0.05）, and those of S-100 protein were 100% and 72.2%. The sensitivities of Langerin, CDla and S-100 protein in the LCH group were 100%, 95.65% and 100%, and their specificities were 100%, 94.4% and 27.8%, respectively. Conclusion Langerin is a highly sensitive and specific immunomarker of LCH, and useful for the diagnosis and differential diagnosis of LCH.