【摘要】 目的探讨朗格汉斯细胞组织细胞增生症(LCH)临床病理特点、免疫表型及预后特点。方法分析6例儿童LCH的临床资料及组织病理学特点,同时用免疫组化染色观察LCH的免疫表型,并结合文献复习。结果男性4例,女性2例,年龄最小23 d,最大13岁。组织学改变:受累器官内朗格汉斯细胞弥漫增生,细胞中等偏大,核呈卵圆形、肾形,可见核折叠。免疫组化:朗格汉斯细胞S-100、CD68和CD1α(+),LCA、CK、CD3、CD5、CD20(-)。结论 LCH临床表现复杂,诊断需要结合临床表现、影像学检查及组织病理学检查。该病患儿预后差别较大,取决于发病年龄、受累器官多少、器官功能受损严重性。更多还原

【Abstract】 Objective To study the clinicopathologic characteristics, immunophenotype and prognosis of Langerhans cell histiocytosis(LCH). Methods Clinical and pathological features were studied in 6 cases of children with LCH,while the immunohistochemical staining was performed to observe the LCH immune phenotype, and combined with the literatures review. Results There were 4 males and 2 females. The youngest was 23-day-old and the oldest was 13 years. Histological changes included: diffused distribution of Langerhans cells, the cells were medium to relatively large, the nucleuses were round, oval, bean-like or folded in shape. Immunohistochemical staining showed that Langerhans cells expressed S-100, CD68 and CD1α, but been negative for LCA, CK, CD3, CD5 and CD20. Conclusion LCH presents complex clinical symptoms. The diagnosis needs a combination of clinical features, image examination and histopathological characters. The prognosis of LCH is various, depending on the age, the number of infiltratedorgans and the function of infiltrated organs.更多还原