Yao X F, Wang X, Wang L, Fu L B, Zhang N, Zhang M, He L J
Department of Pathology, Beijing Children Hospital, Beijing 100042, China.
Zhonghua Bing Li Xue Za Zhi. 2019 Jan 8;48(1):17-21. doi: 10.3760/cma.j.issn.0529-5807.2019.01.004.
To investigate the clinicopathological and ultrastructural characteristics of Langerhans cell histiocytosis (LCH) in children. A total of 345 cases of LCH from the Department of Pathology, Beijing Children Hospital from January 2012 to March 2016 were investigated by hematoxylin-eosin stain, EnVision immunohistochemistry and transmission electron microscopy. The rate of primary clinical diagnosis of LCH in children was 46.0%(210/457). Among 345 patients of LCH, 213 were male and 132 were female, the male to female ratio was 1.6∶1.0, and the median age was 21 months (range from 2 days after birth to 13.3 years). There were total 597 lesions, including bony lesions (258, 43.2%), skin lesions (206, 34.5%) , followed by lymph node (16, 2.7%), lung (28, 4.7%), liver (25, 4.2%) and head-neck (50, 8.4%). Single organ system LCH (SS-LCH) was seen in 295 cases (85.5%) and 50 cases (14.5%) presented with multiple organ system involvement LCH (MS-LCH). There was no significant difference in age and gender between SS-LCH and MS-LCH groups. Regarding sites, more lesions were seen in bone and skin in SS-LCH group, in contrast lymph node, lung, liver and head-neck involvements were often seen in MS-LCH group. Immunohistochemically, the expression of CD1a and Langerin was seen in 99.7% (341/342) and 98.8% (338/342) of the cases respectively. The diagnostic rates by light and transmission electron microscopy were 98.8% (341/345) and 97.4% (112/115) respectively (0.05). LCH of children occurs predominantly in SS-LCH pattern, frequently involving bone, skin, lymph node, lung and liver and other sites with unique histopathological, immunophenotypical and ultrastructural features. Accurate diagnosis relies on the morphology, immunophenotype and ultrastructural features. Further refinement of specimen processing may improve the accuracy of pathological diagnosis.
探讨儿童朗格汉斯细胞组织细胞增多症（LCH）的临床病理及超微结构特征。对2012年1月至2016年3月北京儿童医院病理科的345例LCH病例进行苏木精-伊红染色、EnVision免疫组化及透射电镜检查。儿童LCH的初步临床诊断率为46.0%（210/457）。345例LCH患者中，男性213例，女性132例，男女比例为1.6∶1.0，中位年龄为21个月（范围从出生后2天至13.3岁）。共有597处病灶，包括骨病灶（258处，43.2%）、皮肤病灶（206处，34.5%），其次为淋巴结（16处，2.7%）、肺（28处，4.7%）、肝（25处，4.2%）和头颈部（50处，8.4%）。单器官系统LCH（SS-LCH）295例（85.5%），50例（14.5%）为多器官系统受累LCH（MS-LCH）。SS-LCH组和MS-LCH组在年龄和性别上无显著差异。就部位而言，SS-LCH组骨和皮肤病灶较多，而MS-LCH组常出现淋巴结、肺、肝和头颈部受累。免疫组化方面，CD1a和Langerin的表达分别见于99.7%（341/342）和98.8%（338/342）的病例。光镜和透射电镜的诊断率分别为98.8%（341/345）和97.4%（（112/115）（P>0.05）。儿童LCH主要以SS-LCH形式出现，常累及骨、皮肤、淋巴结、肺和肝等部位，具有独特的组织病理学、免疫表型和超微结构特征。准确诊断依赖于形态学、免疫表型和超微结构特征。进一步优化标本处理可能提高病理诊断的准确性。
