单中心和多中心型Castleman病的诊疗对比体会  

Comparative Experience in Diagnosis and Treatment of Unicentric and Multicentre Castleman Disease

作　　者：周芳[1] 覃宝珍[1] 贾浩 方锃 苏曦 李朋[2] ZHOU Fang;QIN Bao-zhen;JIA Hao;FANG Zeng;SU Xi;LI Peng(Department of Hematology,Peking University Shenzhen Hospital,Shenzhen 518036,Guangdong Province,China;Department of Breast and Thyroid Surgery,Peking University Shenzhen Hospital,Shenzhen 518036,Guangdong Province,China)

机构地区：[1]北京大学深圳医院血液内科,广东深圳518036 [2]北京大学深圳医院乳腺甲状腺外科,广东深圳518036

出　　处：《罕少疾病杂志》2025年第4期139-141,共3页Journal of Rare and Uncommon Diseases

基　　金：深圳市医学重点学科建设经费资助(No.SZXK017)。

摘　　要：目的对比单中心型与多中心型Castleman病在临床诊断、治疗及预后方面的差异,以实现临床精准诊疗。方法回顾性分析1例单中心型和1例多中心型Castleman病患者的诊疗流程及预后情况。结果单中心型Castleman病患者主要临床表现为颈部肿块,经手术完整切除后,定期随访4年未见复发;多中心型Castleman病患者以副肿瘤性天疱疮起病,全身症状及器官损害症状显著,且合并阻塞性细支气管炎,在化疗过程中因发生呼吸衰竭而中断治疗,预计预后较差。结论单中心型Castleman病通常表现为单个区域淋巴结肿大,通过完整手术切除可获得良好预后。多中心型Castleman病则累及全身多个区域淋巴结,其治疗原则为激素联合生物治疗及化疗的综合性全身治疗,手术仅用于诊断性活检。此外,多中心型患者可合并闭塞性细支气管炎,预后相对较差。Objective To compare the differences in diagnosis,treatment,and prognosis between unicentric and multicentre Castleman disease in order to implement accurate diagnosis in clinical practice.Methods A retrospective analysis of the diagnosis,treatment,and prognosis of a single case of unicentric and multicentre Castleman.Results The main clinical manifestation of unicentric Castleman disease patient is a cervical mass,which has been regularly followed up for 4 years without recurrence after surgical resection;the multicenter Castleman patient developed paraneoplastic pemphigus with significant systemic and organ damage symptoms,complicated with obstructive bronchiolitis,and the chemotherapy process was interrupted due to respiratory failure.The prognosis is expected to be poor.Conclusion The clinical manifestation of unicentric Castleman disease is a single regional lymph node enlargement,with a good prognosis after complete surgical resection.Multicentre Castleman involves multiple regional lymph nodes throughout the body,and the treatment principle is glucocorticoids,biological therapy and chemotherapy.The purpose of surgery is only diagnostic biopsy.It can be associated with obliterative bronchiolitis and has a poor prognosis.

关 键 词：单中心型Castleman病 多中心型CASTLEMAN病 诊疗 预后 

分 类 号：R55[医药卫生—血液循环系统疾病]