Castleman病临床诊治分析  

Clinical features analysis of Castleman disease patients

作　　者：顾史洋 魏征 季丽莉 王志梅 庄静丽 程志祥 王伟光 袁玲 承璐雅 柯杨 刘澎 GU Shi-yang;WEI Zheng;JI Li-li;WANG Zhi-mei;ZHUANG Jing-li;CHENG Zhi-xiang;WANG Wei-guang;YUAN Ling;CHENG Lu-ya;KE Yang;LIU Peng(Department of Hematology,Zhongshan Hospital,Fudan University,Shanghai 200032,China)

机构地区：[1]复旦大学附属中山医院血液科,上海200032

出　　处：《中国临床医学》2022年第4期610-614,共5页Chinese Journal of Clinical Medicine

摘　　要：目的分析Castleman病(Castleman disease,CD)的病理及临床特征,以及诊治方面的特点。方法回顾性分析95例病理确诊为CD患者的基线临床资料、实验室指标、病理亚型、治疗方案及生存资料,分析患者病理、临床及诊治特点。结果CD患者的病理亚型以透明血管型(HV)较多(56/95);单中心型(UCD)较多(69/95),21例患者为特发性多中心型Castleman病-非特指型(iMCD-NOS)。UCD及MCD患者间病理亚型、病灶最大径、B组症状、脾大、浆膜腔积液发生率及多项实验室指标差异有统计学意义(P<0.05)。39例(41.1%)患者存在淋巴细胞绝对值降低。UCD及无症状性MCD(aMCD)患者未行全身药物治疗;iMCD患者均接受激素、免疫调节剂、利妥昔单抗、化疗药物中的一种或多种全身治疗。CD患者的3年无进展生存率(PFS)为78.8%,预测3年总生存率(OS)为92.2%;MCD患者3年OS为78.2%,5年OS为70.2%。结论CD患者合并脾大、浆膜腔积液等情况的比例较低,常合并淋巴细胞绝对值降低;MCD患者5年预测OS优于既往报道。Objective To analyze clinical pathological and therapeutic features of Castleman disease(CD).Methods Totally,95 patients were enrolled and their clinical features,lablatory results,pathological features,treatment and survival data were collected.Results Among the CD patients,hyaline vascular pathological subtype accounted for 58.9%(56/95);Uni-center CD(UCD)accounted for 72.6%(69/95)while muitl-center CD(MCD)accounted for 27.4%(26/95).Twenty-one patients had idiopathic MCD-not otherwise specified(iMCD-NOS)subtype.There were significant differences in the pathological subtype,maximum diameter of lesions,the ratios of B symptoms,splenomegaly,and serous effusion,and several laboratory factors between UCD and MCD subgroup(P<0.05).Laboratory examination showed lymphocytopenia happened in 39(41.1%)patients.UCD and asymptomatic MCD(aMCD)patients were free from systemic medication,while iMCD patients were undertaken systemic medication including glucocorticoid,immunomodulatory drugs,rituximab and/or chemotherapy.For all the patients,estimated 3-year progression-free survival(PFS)and overall survival(OS)rates were 78.8%and 92.2%;While for MCD subgroup,estimated 3-year OS and 5-year OS rates were 78.2%and 70.2%.Conclusions The proportions of splenomegaly and serous effusion are low in CD patients,while the lymphocytes decreases,and estimated 5-year OS rate in MCD patients is better than previously reports.

关 键 词：CASTLEMAN病 特发性多中心型Castleman病-非特指型 淋巴细胞减少症 治疗 生存率 

分 类 号：R551.2[医药卫生—血液循环系统疾病]