Department of Pathology, Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai 200092, China.
World J Pediatr. 2010 Aug;6(3):255-9. doi: 10.1007/s12519-010-0205-0. Epub 2010 Jun 12.
Langerhans cell histiocytosis (LCH) is a rare disease that is characterized by abnormal proliferation of pathological Langerhans cells (LCs). In this study, a total of 108 pediatric patients with LCH of bone were evaluated retrospectively for illustrating the clinicopathologic features of this disease, with a goal of improving the diagnosis, treatment, and prognosis.
A retrospective study was based on the clinical records and pathological data of 108 patients (13 days to 12 years of age) with LCH of bone from a single hospital. Hematoxylin-eosin stain and immunohistochemical stain were applied. The follow-up was conducted to June 2008.
The peak age of the patients ranged between 3 years and 6 years (80.6%, 87/108), and male gender predominated. The most common clinical presentation was local pain, and the imaging findings commonly showed an isolated lytic lesion in the bone. Of the 108 patients, 79 (73.1%) had single bone involvement, 27 (25.0%) had multi-bone involvement (with or without related skin involvement), and 2 (1.8%) had multisystem involvement. Histologically, all the lesions revealed abnormal proliferation of pathological Langerhans cells along with an admixture of eosinophils, lymphocytes, and other inflammatory cells. The LCs have similar shape and are positive for cluster of differentiation 1a (CD1a) (100.0%, 60/60), S100 (90.0%, 54/60), CD68 (41.7%, 25/60), lysozyme (Lys) (40.0%, 24/60), and macrophage antigen compound (MAC) 387 (30.0%, 18/60); cytokeratin (CK) and epithelial membrane antigen (EMA) were negative. The overall survival rate was 98.0% at a median follow-up of 5 years.
LCH of bone in children is predominant in males and usually shows as an isolated lytic lesion. Histologically, the lesions reveal abnormal proliferation of pathological Langerhans cells, admixed with various types of inflammatory cells. The patients have a good prognosis, except those with multi-system involvement.
朗格汉斯细胞组织细胞增生症（LCH）是一种罕见疾病，其特征为病理性朗格汉斯细胞（LC）的异常增殖。本研究通过回顾性分析 108 例儿童骨朗格汉斯细胞组织细胞增生症患者的临床病理特征，旨在提高该病的诊断、治疗及预后水平。
本研究采用单中心回顾性研究方法，收集 108 例（年龄 13 天至 12 岁）骨朗格汉斯细胞组织细胞增生症患儿的临床及病理资料，进行苏木素-伊红（HE）染色和免疫组织化学染色。随访至 2008 年 6 月。
患儿发病年龄高峰为 3 岁至 6 岁（80.6%，87/108），男童多见。最常见的临床表现为局部疼痛，影像学表现多为孤立性溶骨性骨病变。108 例患儿中，79 例（73.1%）为单发骨病变，27 例（25.0%）为多骨病变（伴或不伴相关皮肤病变），2 例（1.8%）为多系统病变。组织病理学检查示所有病变均可见病理性朗格汉斯细胞异常增生，伴嗜酸性粒细胞、淋巴细胞及其他炎细胞浸润。LC 形态相似，均表达 CD1a（100.0%，60/60）、S100（90.0%，54/60）、CD68（41.7%，25/60）、溶菌酶（Lys）（40.0%，24/60）和 MAC387（30.0%，18/60），但不表达细胞角蛋白（CK）和上皮膜抗原（EMA）。中位随访 5 年时，患儿总体生存率为 98.0%。
儿童骨朗格汉斯细胞组织细胞增生症以男性多见，多表现为孤立性溶骨性骨病变。组织病理学检查示病变由病理性朗格汉斯细胞异常增生，伴多种炎细胞浸润。患儿预后较好，除多系统受累者外。
