成人朗格汉斯细胞组织细胞增生症5例临床病理分析  

Langerhans cell histiocytosis in adults:a clinicopathologic analysis of 5 cases

作　　者：魏丽[1] 杨冬梅[1] 李雪[1] 

机构地区：[1]首都医科大学附属北京同仁医院病理科,北京100730

出　　处：《诊断病理学杂志》2011年第6期420-423,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨成人朗格汉斯细胞组织细胞增生症(LCH)的临床病理学特征、免疫表型及预后。方法分析5例成人LCH的临床病理学特点,同时用免疫组化染色观察LCH的免疫表型,采用原位分子杂交方法检测病变组织中EBV编码的小RNA(EBER)。结果 5例成人LCH均为男性,发病年龄21～29岁。组织学改变为朗格汉斯细胞弥漫分布,细胞中等至偏大,胞质淡染至嗜酸,核呈圆形、卵圆形、咖啡豆样或不规则形,可见核沟和凹陷,间质富于小血管和纤维母细胞,并见不同程度的嗜酸性粒细胞、淋巴细胞及中性粒细胞浸润。免疫组化示朗格汉斯细胞S-100、CD68和CD1a(+),p53不同程度(+),Ki-67增殖指数5%～15%。原位分子杂交示病变组织中EBER均(-)。结论成人LCH可以发生于颅眶、外耳道、肛周及背部皮肤,手术切除结合其他治疗,预后较好。Objective To investigate the clinicopathologic features,immunophenotype and prognosis of Langerhans cell histiocytosis（LCH） in adults.Methods Analysis of 5 cases of clinical and pathological features of adult LCH,while the SP immunohistochemical staining were performed for LCH immune phenotype.The encoded small RNA was detected by in situ hybridization.Results The 5 adult patients with LCH were male,aged 21-29 years old.Histological changes included： diffused distribution of Langerhans cells,the cells medium to relatively large,lightly stained to eosinophilic cytoplasm,nuclear were round,oval,bean-like or irregular in shape,showing nuclear grooves and depressions,inter-rich quality of the small blood vessels and fibroblasts,and the different number of eosinophils,lymphocytes and neutrophil infiltration.Langerhans cells expressed S-100,CD68 and CD1a,expressed varying degrees of P53,and Ki-67 proliferation index showed 5%~15%.In situ hybridization showed： The cases were negative for EBER.Conclusions Adults LCH can occur in cranio-orbital cavity,auditory canal,perianal and back skin.The prognosis is better when surgical resection is combined with other treatments.

关 键 词：成人 朗格汉斯细胞组织细胞增生症 临床病理 

分 类 号：R733.1[医药卫生—肿瘤]