朗格汉斯细胞组织细胞增生症14例临床病理分析

Clinicopathologic analysis of Langerhans 'cell histiocytosis:14 cases report

目的探讨朗格汉斯细胞组织细胞增生症（LCH）的临床病理特征、免疫表型、诊断与鉴别诊断。方法分析14例LCH的临床特征,应用HE染色及免疫组化技术观察其病理学特征,并复习文献。结果本组病例中男11例,女3例,年龄9个月~52岁,累及骨13例（颅骨8例,下肢骨3例,椎骨2例）,累及皮肤2例,光镜下可见朗格汉斯细胞（LCs）呈卵圆形,核不规则、有核沟和核折叠,核仁不明显,核膜薄,细胞质中等量,略呈嗜酸性,伴多少不等的嗜酸性粒细胞浸润。免疫组化表达CDIa、S-100均阳性,Langerin阳性12例（（2例未做）,CD68阳性9例,LCA、CD3和CD20均（-）,Ki67阳性率10%~20%。结论 LCH的临床表现复杂,从单系统病变到多系统病变,好发于骨,确诊需取活检进行病理学检查;LCs的CD1a、S-100和Langerin的阳性表达对于LCH的诊断与鉴别诊断有重要意义。

Objective To explore the clinicopathological features,immunophenotypes,diagnosis and differential diagnosis of Langerhanscell histiocytosis（LCH）.Methods Analysis of the clinical features of 14 LCH cases were performed,HE staining and immunohistochemical techniques were applied to observe the pathological features,the relative literatures were also reviewed.Results The patients consist of 11 males and 3 females,aged from 9 months to 52 years,13 cases of LCH were involved in bones（8 in skull,3 in lower limb,2 in vertebra）,and 2 in skin.Light microscopy showed oval Langerhans cells（LCs）with irregular,grooved or folded nuclei,inconspicuous nucleoli,thin nuclear membranes,moderately abundant and slightly eosinophilic cytoplasm,accompanied with variable numbers of eosinophils infiltration.Immunohistochemical expressions of CD1a and S-100 were positive in all cases,12 cases expressed Langerin（2 cases didnt examined）,9 cases expressed CD68,LCA,CD3 and CD20 were negative in all cases,ki67 positive rate was 10%to 20%.Conclusion The clinical manifestation of LCH varies from a single system lesion to multi-system disease,and often occurs in bone,the diagnosis should be confirmed by pathological examination of biopsy;CD1a,S-100 and Langerin positive expressions in LCs have important significance for diagnosis and differential diagnosis of LCH.