摘要
目的 探讨朗格汉斯细胞组织细胞增生症（LCH）的临床病理特征及生物学行为.方法 对4例LCH的临床病理特征和免疫组化结果结合相关文献进行分析.结果 3例男性,1例女性,发病年龄7～45岁,平均17.8岁.病变部位分别为胫骨、肱骨、额骨,双侧腋下及阴囊皮肤.镜下可见Langerhans细胞呈不同程度的增生,伴有数量不等的嗜酸性粒细胞、淋巴细胞、浆细胞、中性白细胞及多核巨细胞.免疫组化标记4例Langerhans细胞表达CD1a、S100,3例表达CD68.随访结果显示3例骨LCH无复发,1例皮肤LCH半年后复发.结论 LCH是具有特征的Langerhans细胞异常增生的少见疾病,对其确诊必需结合临床特征、病理形态、免疫表型,且需与炎症、肿瘤及其他组织细胞增生症相鉴别.大多数发生于骨和皮肤的LCH患者预后良好.LCH预后与病变发生部位、累及范围、有无器官功能障碍及年龄有关.
Objective To investigate the clinicopathologic features and prognosis of langer- hans cell histiocytosis （LCH）. Methods The elinicopathologic features and results of immunohisto- chemistry of the four LCH cases and relevant literature were retrospectively analyzed. Results There were 3 males and 1 female, and the age ranged from 7 to 45 years with a mean of 17.8 years. The areas were mainly located in tibia, humerus, frontal bone and skins. Microscopically, LCH was characterized by abnormal proliferation of Langerhans cell, mixed with various types of inflammatory cells. Immunohistochemically, langerhans cells were positive for CD1 a and S100 protein of all cases, and positive for CD68 of 3 cases. The follow-up showed that 1 patient had skin involvement. Conclu- sions LCH is a rare disease that is characterized by abnormal proliferation of pathological Langer- hans ceils. The diagnosis of LCH needs clinical data, HE staining and immunohistochemistry. It needs to be distinguished from inflammations, tumors and other histiocytosis diseases. Most patients with bone or skin involvement have a good prognosis. The prognosis of LCH must depend on the area, the number of infiltrated organs, the function of infiltrated organs and the age.
出处
《实用临床医药杂志》
CAS
2013年第19期50-53,共4页
Journal of Clinical Medicine in Practice
