成人朗格汉斯细胞组织细胞增生症1例并文献复习

Adult Langerhans Cell Histiocytosis A Case Report and Literature Review

目的探讨成人朗格汉斯细胞组织细胞增生症(LCH)的临床、组织病理学、免疫表型。方法报告1例罕见的皮损类似播散性黄瘤的成人朗格汉斯细胞组织细胞增生症,并结合文献分析其临床及病理特征。结果该病例表现有皮肤、骨骼、垂体三系统损害。先后经过2次皮损活检及1次骨骼活检,皮损组织学特点为真皮全层弥漫分布的组织细胞,其CD1a表达阴性而S-100表达阳性,受损骨骼组织学显示典型嗜酸性肉芽肿表现,CD1a,S-100均表达阳性。结论成人朗汉斯细胞组织细胞增生症罕见,误诊率高。应结合多系统临床表现及组织学特征、免疫表型综合分析。

Objective To investigate the clinical and pathological,immunophenotypical microscopical features of lange- rhans cell histiocytosis （LCH）. Methods An adult LCH was reported, accompanied by accompanied by lesions similar to xanthoma and osteolysis. The clinicopathological features were summarized by literature review. Results The case showed three system damages of skin, bones, and pituitary. Pathological exa mina- tion of skin lesion showed diffuse histocyte infiltration, with positive S-100 and negative CD10a. Pathological examination of femur showed typical eosinophilic granuloma, with positive S-100 and positive CDIOa. The lesion removed from femur was positive for S-100 and CDla. Conclusion Adult langerhans cell histiocytosis is extremely rare. The clinical presentation of LCH is highly variable.