骨朗格汉斯细胞组织细胞增生症3例临床病理分析

Clinicopathologic Analysis of 3 Cases with Langerhans Cell Histiocytosis of Bone

目的探讨骨朗格汉斯细胞组织细胞增生症（langerhans cell histiocytosis，LCH）的临床病理特征、免疫表型、鉴别诊断及生物学行为。方法对3例骨LcH临床特征、组织学形态和免疫组织化学结果结合相关文献进行分析。结果2例男性，1例女性，发病年龄7～12岁（平均8．7岁）。病变部位分别位于肱骨、胫骨及额骨；镜下示Langerhans胞异常增生，伴有数量不等的嗜酸性粒细胞、淋巴细胞、中性白细胞及多核巨细胞；免疫组织化学提示，3例Langerhans细胞弥漫表达CD1a和s100。结论绝大多数发生于骨朗格汉斯细胞组织细胞增生症患者预后较好。其预后与病变部位、累及范围、有无器官功能异常及年龄相关。

Objective To investigate the Clinicopathologic features, immunophenotype, differential diagnosis and prognosis of Langerhans cell histiocytosis of bone. Methods Three cases of LCH were analyzed by clinical data, HE staining and immunohistochemistry of EnVision technique, and review the related literatures. Results Three cases ranged from 7 to 12 years were 2 males and 1 female （mean 8.7 years）. The sites of 3 cases was localized in tibia, humerus and frontal bone. Microscopically, LCH was characterized by abnormal proliferative of pathological Langerhans cell, with eosinophils, lymphocyte, neutrophils and some multinucleated giant cell. Immunohistochemically, langerhans cells were positive for CDla and S100 protein in all cases. Conclusion The most patients of Bone LCH had a good prognosis, which depended on lesions involving the range, with or without organ dysfunction and age.