摘要目的 增加对Castleman病(CD)的临床特征、病理特点及预后的了解,并对其治疗方案进行探讨.方法 对2003年1月至2008年10月经病理诊断的26例CD患者的临床特征、病例特点、治疗方案、疗效等进行回顾性分析.结果 26例患者男女比例为1∶1.6,年龄6～77岁.临床分类局灶型CD(LCD)20例(76.9%),多中心型CD(MCD)6例(23.1%),2例存在反应性浆细胞增多,1例出现自身免疫紊乱,1例出现弥漫性肺间质改变.病理分类:透明血管型(HV)型24例(92.3%),浆细胞型(PC)型2例(7.7%).手术完全切除19例(73%)均为LCD,部分切除3例(11.5%),化疗4例(15.4%),方案采用COP、CHOP及ECHOP.随访至2008年10月,26例均生存,平均生存期已达到5年.结论 CD为临床少见疾病,女性发病率略高于男性,无年龄差别.多以无痛性淋巴结肿大首发,淋巴结肿大单发多见,多发者多同时伴有全身症状.病理为诊断金标准.HV型较PC型多见.PC型可同时伴有反应性浆细胞增多,并可合并自身免疫紊乱.LCD型经手术治疗后多可痊愈.MCD型可应用化疗,以COP为主的化疗多可使淋巴结明显减小,但对浆细胞增多等改变不明显.

abstractsObjective To investigate the clinical, pathological features of Casfleman's disease (CD)and evaluate the treatment and prognosis. Methods Twenty six cases of CD diagnosed by pathological examination from January 2003 to October 2008 were analyzed retrospectively. Results The ratio of male to (76.9 %) patients are local CD(LCD) and six (23.1%) are multicentric CD (MCD). Two of them show the inereaseot reactive plasma cells in bone marrow. One patient was complicated with autoimmunal disorder. One patient complicated diffused Interstitial lung change. According to the pathological classification, twenty four patients (92.3 %) are hyaline vascular type (HV), and two (7.7 %) are plasma cell type (PC). For treatment,ninety patients (73 %) that totally LCD type accepted completely resection, three patients (11.5 %) accepted uncompleted resection, four patients (15.4 %) accepted chemotherapy COP, CHOP, ECHOP. Follow-up toOctober 2008, all the patients are alive and average survival time is 5 years. Conclusion CD is a rare lymphoproliferative disorder, there are slightly more female patients than male in morbility, no age difference is found in CD. Lymphomegaly and no pain is the common symptoms. Single lymphomegaly is more often,system symptoms are commonly occurred in MCD. Pathological examination is the golden standard in diagnosis. HV type is more than PC. PC type can complicated reactive plasma cells and increase autoimmunal disorder. The patients of LCD would be cured with completely excision, and chemotherapy such as COP will significantly reduced the lymphomegaly but lightly to the systematical symptoms for MCD type.