摘要
目的探讨儿童Langerhans细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)的临床特点、病理形态学及免疫表型特征,并分析其与预后的关系。方法分析38例儿童LCH的临床及病理资料,其中18例做了免疫组化染色。结果在38例儿童LCH中,单发为30例(占78·9%),多发为8例(占21·1%)。病变发生于骨组织34例(占89·5%)(其中1例伴有皮肤病变),发生于皮肤组织3例,发生于淋巴结1例。镜下病变主要由Langerhans细胞及嗜酸性粒细胞组成。免疫组化结果显示Langerhans细胞阳性表达CD1a为100%(13/13),S-100蛋白为88·2%(15/17),vimentin为90·0%(9/10),CD68为81·8%(9/11),Mac387为30·0%(3/10),lysozyme为40·0%(4/10),CK、EMA、CD45均阴性。结论儿童LCH最多发生于骨组织,在病理学上具有特殊的形态学表现以及免疫组化表型。疾病的预后与病理分型及临床分级有关。
Purpose To investigate the clinicopathological and immunohistochemical characteristics of Langerhans cell histiocytosis in children and to explore those relationship with prognosis. Methods The clinical presentation, histological features, immumohistochemical characteristics were analyzed. Results Thirty-eight cases presented as solitary （30/38） or muhifocal （8/38） defects. Among them, 34 cases were situated in bone, 3 cases in skin and 1 case in lymph node. Microscopically, it was characterized by the idiopathic proliferation of Langerhans cell and eosinophils infiltration. 18 cases of them were immunostained ,and the Langerhans cells expressed as CD1a＋ （13/13）, S-100＋ （15/17）, vimentin＋ （9/10）, CD68＋ （9/11）, Mac387＋ （3/10）, lysozyme＋ （4/10）, hut CK - , EMA - , and CD45 -. Conclusions Children＇ s Langerhans cell histiocytosis occours predominantly in bone. It has specially clinicopathological and immunohistochemical characteristics, and its prognosis is related with both histological classification and clinical grading.
出处
《临床与实验病理学杂志》
CAS
CSCD
北大核心
2006年第3期298-301,共4页
Chinese Journal of Clinical and Experimental Pathology
