作　　者： (甄俊锋）; (张路）; (曹欣欣）; (冯俊）; (周道斌）; (林松柏）; (李剑）;

机构地区： 中国医学科学院北京协和医学院北京协和医院国际医疗部,北京100730

出　　处： 《中国医学科学院学报》 2017年第4期492-498,共7页

摘　　要： 目的探讨合并副肿瘤天疱疮及闭塞性细支气管炎的单中心型Castleman病的临床特点,以提高临床医师对此类疾病的认识。方法汇总分析北京协和医院收治及文献报道的同时合并副肿瘤天疱疮及闭塞性细支气管炎的单中心型Castleman病病例的临床资料、治疗及生存转归数据。结果共纳入23例患者,男∶女=11∶12,中位年龄31岁(13~56岁)。单中心型Castlman病的病理类型以透明血管型为主(91.4%),病灶部位以腹盆腔病灶多见(69.6%),26.3%表现为巨块型病灶。患者多以副肿瘤天疱疮症状首诊(90.0%),多在Castleman病手术后逐渐出现闭塞性细支气管炎表现。平均随访时间为27.5个月(1~135个月),患者的中位总生存时间为36.0个月。闭塞性支气管炎所致的呼吸衰竭是主要死亡原因(91.7%)。结论重视副肿瘤天疱疮症状的早期识别、积极寻找并治疗潜在的Castleman病,有利于避免并发症的出现和死亡。 Objective To investigate the clinical features of unicentric Castleman＇s disease（UCD）with paraneoplastic pemphigus（PNP） and bronchiolitis obliterans（BO）.Method Data of UCD patients with PNP and BO from Peking Union Medical College Hospital were retrospectively analyzed,along with literatures review.Results Totally 23 cases（11 males and 12 females） were enrolled.The median age was 31 years（13-56years）.The most common pathological type was hyaline-vascular variant（91.4%）,and most tumors located in abdominopelvic cavity（69.6%）.Considerable cases presented bulky masses（26.3%）.Most cases were first diagnosed on presentation with the symtoms of PNP（90.0%）.BO was characterized by progressive dyspnea after excision of CD lesions.The average follow-up duration was 27.5 months（1-135 months）.The median overall survival time was 36.0 months（95% CI=13.9-58.1）.Respiratory failure was the dominant cause of death（91.7%）.Conclusions PNP should be considered among those patients with specific oral or cutaneous lesions.Earlier diagnosis and treatment of latent UCD are important for reducing complications and deaths.

关 键 词： 病 副肿瘤天疱疮 闭塞性细支气管炎