伴发副肿瘤性天疱疮的Castleman瘤——附10例报告  

Castleman Tumor in Association with Paraneopiastic Pemphigus-A Report of 10 Cases

作　　者：朱学骏[1] 王京[1] 陈喜雪[1] 王仁贵[2] 张澜波[3] 李挺[4] 王爱平[1] 杨淑霞[1] 涂平[1] 李若瑜[1] 吴艳[1] 杨海珍[1] 季素珍[1] 

机构地区：[1]北京大学第一医院皮肤性病科,100034 [2]北京大学第一医院 医学影像科,100034 [3]北京大学第一医院普通外科,100034 [4]病理科,北京大学第一医院100034

出　　处：《中华皮肤科杂志》2005年第12期745-747,共3页Chinese Journal of Dermatology

基　　金：国家自然科学基金资助项目(30371292);2003年度高校博士点专项科研基金(20030001021)

摘　　要：目的了解Castleman瘤伴发副肿瘤性天疱疮的临床特点。方法分析总结我科诊治的10 例Castleman瘤伴发副肿瘤性天疱疮的临床特点和治疗方法。结果 Castleman瘤为我国最常见伴发副肿瘤性天疱疮的肿瘤,其临床表现、皮损和肿瘤的病理学特点、CT及免疫学特点都较独特。肿瘤切除手术中给予静脉滴注丙种球蛋白,对降低术后呼吸道症状的出现,使患者早日康复是有益的。结论由于肿瘤直接产生抗体致病,因此早期诊断、早期切除肿瘤,是成功治疗本病的关键。Objective To obtain a better understaning of the clinical features of Castleman tumor- associated paraneoplastic pemphigus. Methods The clinical features and therapy of 10 cases of this disease, diagnosed in the Department of Dermatology of Peking University First Hospital were analyzed. Results Castleman tumor was shown to be the most common neoplasm associated with paraneoplastic pemphigus in China. The clinical presentations, histopathologic characteristics, CT scan findings, and immunologic features were all unique. The early diagnosis and removal of the Castleman tumor arc crucial for the treatment of this tumor-associated autoimmune disease. Conclusions Because Castleman tumor is directly related to the induction of autnimmunity, early diagnosis and prompt removal of the tumor are essential to the management of this disease.

关 键 词：副肿瘤性天疱疮 巨淋巴结增生 

分 类 号：R758.66[医药卫生—皮肤病学与性病学] R758.23[医药卫生—临床医学]