作者单位：511480 广州，广州市南沙区榄核医院(赵银英)；510630 广州，中山大学附属第三医院呼吸内科(周宇麒，冯定云，杨海玲，刘翠婷)

    Castleman 病一例并文献复习

    赵银英周宇麒冯定云杨海玲刘翠婷

    【摘要】目的提高对Castleman 病的临床认识。方法报道1例Castleman 病的典型病例，并以“Castleman病”为检索词在万方数据知识服务平台检索相关文献，结合检索结果分析Castleman 病的临床表现、病理及治疗特点。结果该例45岁男性患者以干咳2年、胸闷半年收入院，入院后查胸部CT示双肺弥漫性网格状改变，纵隔、双肺门及双侧腋窝多发肿大淋巴结影，考虑为弥漫性肺间质纤维化，经腋窝淋巴结活组织检查(活检)确诊为Castleman病，行化学治疗后病情稳定。万方知识数据平台中共收集200例Castleman病的病例，男∶女为1.27∶1，各年龄段均可发病；多中心型85例，局灶型115例，误诊13例，有合并症51例；在接受治疗的187例患者中，手术治疗85例，化学治疗58例，手术联合化学治疗12例，单纯糖皮质激素(激素)治疗10例，其他方式治疗22例；随访166例，治愈89例，好转61例，治疗效果差12例，死亡4例。结论Castleman病无特征性临床表现，多有典型的病理组织学特点，容易误、漏诊，确诊需结合临床与病理学表现，该病经化学治疗可获缓解。

    【关键词】Castleman病；临床表现；病理；误诊

    DOI：10.3969/g.issn.0253-9802.2015.06.014

    通讯作者，周宇麒，E-mail：zzyyqqcj7605@163.com

    收稿日期：(2015-03-04)

    Castleman’s disease: one case report and literature reviewZhaoYinying,ZhouYuqi,FengDingyun,YangHailing,LiuCuiting.DepartmentofRespiratory,theThirdAffiliatedHospitalofSunYat-senUniversity,Guangzhou510630,China

    Correspondingauthor,ZhouYuqi,E-mail：zzyyqqcj7605@163.com

    Abstract【】ObjectiveTo enhance the understanding of clinical manifestations and treatment of Castleman’s disease. MethodsOne typical case of Castleman’s disease was reported and relevant literature was reviewed by searching the keyword “Castleman’s disease” in Wanfang Data. Clinical manifestations, pathological and therapeutic characteristics of Castleman’s disease were analyzed. ResultsA 45 year old male was admitted because of dry cough for 2 years and chest distress for 6 months. After hospitalization, chest CT revealed bilateral pulmonary pathological changes in diffuse grid pattern and multiple lymph node enlargement in the mediastinum, bilateral hilar and axillary, considered as diffuse pulmonary interstitial fibrosis. Axillary lymph node biopsy was performed to confirm the final diagnosis of Castleman’s disease. The symptoms were alleviated after chemotherapy. A total of 200 cases of Castleman’s disease were searched from Wanfang Data with a male to female ratio of 1.27:1 of all ages. Among them, 85 were diagnosed with multicentric Castleman’s disease, 115 with unicentric type, 13 misdiagnosed cases and 51 with other complications. Among 187 receiving treatment, 85 underwent surgery, 58 received chemotherapy, 12 underwent combined therapy of surgery and chemotherapy, 10 were treated with glucocorticoid hormone therapy and 22 with alternative treatment. In total, 166 patients were followed up, 89 were healed, 61 were alleviated, 12 had low clinical efficacy and 4 died. ConclusionsCastleman’s disease was characterized with no specific clinical manifestations. It was mainly manifested with typical histopathological features. It was easily to miss the diagnosis or make a misdiagnosis. The final diagnosis should be confirmed by clinical and pathological manifestations. Castleman’s disease could be alleviated by chemotherapy. ......