先天性自愈性朗格汉斯组织细胞增生症临床病理分析

Congenital self-healig Langerhans cell histiocytosis: a clinicopathological analysis

目的国内首次报道先天性自愈性朗格汉斯组织细胞增生症(CSHLCH)1例,也被称为先天性自愈性网状组织细胞增生症(CSHRH),结合文献探讨其性质、命名、诊断和鉴别诊断。方法对该病例进行临床病理分析及免疫组化观察。结果患者为1个半月的婴儿,出生时发现右下睑一结节性病变,镜下示炎症背景,有淋巴细胞、中性粒细胞及嗜酸性粒细胞等炎细胞浸润,其间可见聚集成堆的单核样组织细胞,胞质丰富,淡伊红或泡沫状,核圆,较大,居中或略偏位,核分裂偶见;免疫表型S-100、CD1a(+)。结论本病少见,易被误诊、漏诊。需与Letterer-Siwe病、肉芽肿性病变等区别。需长期随访。

Objective To report a case of congenital self-healing Langerhans cell hisfiocytosis （CSHLCH）, also called as congenital serf-healing reticulohistiocytosis. Methods The clinicopathologic data and immunohistochemical findings of CSHLCH were analyzed with review of the related articles to expound the entity, terminology, diagnosis and differential diagnoses of this lesion. Results A baby, one and a half month, was born with a nodule at the right lower eyelid. Microscopically, the lesion showed an inflammatory background, mainly lymphocytes, neutrophils and eosinophils. There were aggregated mononuclear histiocytes with abundant eosinophilic or foamy cytoplasm and centrally or httle eccentrically located nuclei. A few mitotic figures could be seen. These mononuclear histiocytoid ceils were positive for S-100 protein and CDIa. Conclusion CSHLCH is a rare variant of Langerhans cell histiocytosis, and often be misdiagnosed. It should be differentiated firm Letterer-Siwe disease and granulomatous disease. A long-term follow-up is suggested.