朗格汉斯细胞组织细胞增生症6例临床病理剖析.docVIP

朗格汉斯细胞组织细胞增生症6例临床病理剖析 　　[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。 　　[关键词] 朗格汉斯组织细胞增生症；病理分析；免疫表型 　　[中图分类号] R733 [文献标识码] A [文章编号] 1673-7210（2015）05（b）-0161-04 　　Clinicopathologic analysis of 6 cases with Langerhans cell histiocytosis 　　QIU Li LIU Yuhong WEN Shouqing WANG Chunfu CHEN Lei GONG Jinqing 　　Baoan Peoples Hospital of Shenzhen City， Guangdong Province， Shenzhen 518101， China 　　[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. 　　[Key words] Langerhans cell histiocytosis； Pathology analysis； Immunophenotyping 　　朗格汉斯细胞组织细胞增生症（Langerhans cell histiocytosis，LCH）是一种因朗格汉斯细胞（Langerhans cell）克隆性弥漫增生浸润，导致器官功能障碍为特征的罕见肿瘤性疾病，曾用名嗜酸性肉芽肿、组织细胞增生症X、Langerhans细胞肉芽肿病等，其发病率约为5/100万，在任何年龄阶段均可发病，但主要患者人群见于低龄儿童，男性较为多见（男女之比约为