67例儿童朗格汉斯细胞组织细胞增生症的临床病理分析

Langerhans Cell Histiocytosis in Children: A Clinical Pathologic Study of 67 Cases

目的探讨儿童朗格汉斯细胞组织细胞增生症(LCH)的临床特点、病理诊断、鉴别诊断及预后等。方法对67例儿童LCH的临床资料、病理学表现和免疫组化结果进行分析。结果在67例儿童LCH中,单发为57例(85%),多发10例(15%),病变发生于骨组织48例(占72%),发生于皮肤组织9例,发生于淋巴结10例。镜下病变主要由朗格汉斯细胞及嗜酸性粒细胞组成。免疫组化结果显示朗格汉斯细胞阳性表达CD1a 100%(63/63),S-100阳性率为100%(63/63),CD68为78%(45/58),Vimentin为100%(9/9),CK、CD20、CD3、EMA、CD45均阴性。结论儿童LCH临床表现复杂,从单系统病变到多系统病变,最多发生于骨组织,确诊需取活检进行病理学检查。单系统病变预后较好,累及重要器官的多系统病变预后较差。

Objective To investigate clinical and histopathological characteristics, diagnostic criteria, differential diagnosis and prognosis of Langerhans cell histiocytosis in children. Methods The clinical, pathological features and immunohistochemical characteristics were studied in 67 cases of Langerhans cell histioeytosis. Results 67 cases presented as solitary （57/67） or multifocal （10/67）. Among them, 48 cases were situated in bone, 9 cases in skin and 10 cases in lymph node. Microscopically, it was characterized by idiopathic proliferation of Langerhans cell and eosinophils infiltration. 63 cases of them were immunostained, and the Langerhans cells expressed as CD1a （63/63）, S-100 （63/63）, CD68 （45/58）, vimentin＋ （9/9）, but negative for CK, CD20, CD3, EMA and CD45. Conclusions The clinical manifestation of LCH varies from a single system lesion to multi-system disease, and often occurs in bone, the diagnosis should be confirmed by pathological examination of biopsy. The patients have a good prognosis, except those with multi-system involvement.