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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 clinicopathological characteristics, immunophenotype and prognosis of adult Langerhans cell histiocytosis (LCH). Methods The clinicopathological features of 5 adult patients with LCH were analyzed. The immunophenotype of LCH was observed by immunohistochemistry. The EBV - encoded small RNA (EBER) was detected by in situ hybridization. Results All 5 adult LCHs were male and their age of onset was 21-29 years old. Histological changes for the Langerhans cells diffuse distribution, the cells are medium to large, light cytoplasm to eosinophilic, nucleus was round, oval, coffee bean-like or irregular shape, showing the nuclear ditch and depression, between Rich in blood vessels and fibroblasts, and see varying degrees of eosinophils, lymphocytes and neutrophil infiltration. Immunohistochemistry showed that Langerhans cells S-100, CD68 and CD1a (+), p53 different degrees (+), Ki-67 proliferation index of 5% to 15%. In situ hybridization showed EBER lesions (-). Conclusion Adult LCH can occur in the cranial orbital, external auditory meatus, perianal and dorsal skin. Surgical resection combined with other treatment, the prognosis is good.