骨朗格汉斯组织细胞增生症八例并文献复习  

Langerhans Cell Histiocytosis of Bone:Report of Eight Cases and Review of the Literature

作　　者：毕娅 吴丹丹 余方颖 方圳鸿 黄波 BI Ya;WU Dandan;YU Fangying;FANG Zhenhong;HUANG Bo(Department of Respiratory and Critical Care Medicine,Affiliated Hospital of Zunyi Medical University,Zunyi,Guizhou 563003,China)

机构地区：[1]遵义医科大学附属医院呼吸与危重症医学科,贵州遵义563003

出　　处：《中国医学科学院学报》2025年第2期325-332,共8页Acta Academiae Medicinae Sinicae

摘　　要：骨朗格汉斯组织细胞增生症是一种原因未明的以CD1a^(+)、CD207^(+)树突状细胞在组织中大量积累为特征的肿瘤属性罕见疾病,发病高峰年龄为1~4岁,发病率为(4~6)/100万,成人发病率为(1~2)/100万。由于其发病率低,临床表现多种多样,影像学表现无明显特异性,为该类肿瘤的明确诊断及早期治疗带来挑战。本文报道8例骨朗格汉斯组织细胞增生症,同时回顾近5年国内外相关文献,对骨朗格汉斯组织细胞增生症的临床特点、病理特征、诊断、治疗及预后进行总结。Langerhans cell histiocytosis of bone is a rare tumor disease characterized by the large accumulation of CD1a^(+)and CD207^(+)dendritic cells in tissues of unknown cause.It mainly occurs in children aged 1-4 years old,with incidences of 4-6 per million in children and 1-2 per million in adults.Due to its low incidence,diverse clinical manifestations,and no obvious specificity of imaging manifestations,the definitive diagnosis and early treatment of this type of tumor are challenging.In this paper,we report 8 cases of Langerhans cell histiocytosis of bone and review the relevant literature published in the past five years to summarize the clinical characteristics,pathological features,diagnosis,treatment,and prognosis of this disease.

关 键 词：骨朗格汉斯组织细胞增生症 诊断 治疗 免疫组织化学 

分 类 号：R365[医药卫生—病理学] R738[医药卫生—基础医学]