Castleman病65例诊治分析

目的 总结Castleman病的临床特点和治疗手段.方法 回顾2005-2012年连续收治的65例Castleman病患者的临床资料,总结分析局灶型Castleman病和多中心型Castleman病在临床表现、发病部位、实验室检查及治疗上的不同.结果 本组42例局灶型Castleman病多见于中青年,常为单发肿瘤,表现为无症状的局部淋巴结增大,病理分型以透明血管型(33/42,78.6％)为主,本组42例患者发现44枚肿瘤,以腹膜后腔(10/44,22.7％)、腹腔(10/44,22.7％)等深部多见,通过手术治疗可治愈.本组23例多中心型Castleman病多见于中年,表现为多发淋巴结肿大,无局部淋巴结疼痛及表面破溃,常伴有发热(9/23,39.1％)、贫血(9/23,39.1％)、脾大,实验室检查多见异常,病理分型主要为浆细胞型(14/23,60.9％)和昆合型(5/23,21.7％),本组23例患者发现66枚肿瘤,以颈部(16/66,24.2％)和股沟区(12/66,l8.2％)等浅表部位多见,主要采用CHOP化疗方案或手术联合CHOP化疗方案,本组21例患者获得完全或部分缓解,2例因化疗引起肺部感染死亡,能否耐受长期化疗是影响预后的重要因素.结论 局灶型和多中心型Castleman病临床特点不同,治疗方法也有差异.局灶型Castleman病通过手术可达到治愈目的;多中心型Castleman病则主要采用CHOP化疗方案,多数预后较好.

Retrospective analysis of 65 Castleman diseases

Objective To explore and clarify the clinical features and management of patients with Castleman disease.Methods The clinicopathological features and therapeutic method of 65 patients with Castleman disease admitted to our hospital continuously from 2005 to 2012 were analyzed retrospectively.The mean follow-up time for the entire series was 40 (15-71) months.Results In contrast to Localized Castleman disease(LCD),Multicentric Castleman disease (MCD) patients were relatively older and exhibit various symptoms and abnormal laboratory tests.We found 44 tumor in LCD group.LCD ones,most of which were hyaline vascular (33/42,78.6％),were more likely originated in retroperitoneum,enterocoelia(10/44,22.7％) or mediastinum(10/44,22.7％).The initial treatment for LCD patients was complete surgical resection and all patients are current alive with no evidence of recurrence.MCD ones always accompanied by fever(9/23,39.1％),anemia(9/23,39.1％)and splenomegaly,and most of MCD patients were plasmacytic variants(14/23,60.9％) or mixed cellular variants (5/23,21.7％).We found 66 tumor in MCD group and they were more likely originated in neck (16/66,24.2％) and groin area (12/66,18.2％).Surgery combined with CHOP-like chemotherapy or CHOP-like chemotherapy alone was the mainstream treatment for MCD ones.21 patients achieved complete remission or partial remission,and only 2 patients died of lung infection.Whether patients could tolerate long-term chemotherapy or not was an important prognostic factor.Conclusions LCD patients had no typical clinical symptoms and could be cured by surgery.Most of MCD patients always had a good prognosis after receiving CHOP-like chemotherapy.