摘要
目的:探讨蛋白酶体抑制剂硼替佐米联合地塞米松方案(BD方案)序贯自体造血干细胞移植(autologous hematopoietic stem cell transplantation,auto-HSCT)治疗POEMS综合征的安全性及疗效的临床观察。方法:回顾性分析2019年2月—2023年11月在甘肃省人民医院确诊的6例POEMS综合征患者的临床特征、诊治方案及随访结果,并结合相关文献进行讨论。结果:6例患者中男1例,女5例,中位年龄为49(37~58)岁。6例患者均接受BD方案化疗至少2个周期,均采用环磷酰胺联合粒细胞集落刺激因子动员,获得中位单个核细胞数3.5×10^(8)/kg和中位CD34+细胞数3.6×10^(6)/kg。后经大剂量马法兰预处理后行auto-HSCT。所有患者化疗及移植期间耐受性好,粒细胞植入中位时间为+10.5d,血小板植入中位时间为+12.5d,无移植相关死亡。中位随访时间34(16~57)个月,总生存率为83%(5/6)。结论:6例POEMS综合征患者经BD方案序贯auto-HSCT后血管内皮生长因子完全达到正常,BD方案序贯auto-HSCT治疗POEMS综合征是一种安全有效的选择。
Objective:To explore the safety and efficacy of proteasome inhibitor bortezomib combined with dexamethasone(BD)regimen followed by autologous hematopoietic stem cell transplantation(auto-HSCT)in the treatment of POEMS syndrome.Methods:The clinical characteristics,diagnosis and treatment regimens and f ollow-up results of 6patients with POEMS syndrome diagnosed in Gansu Provincial Hospital from February 2019 to November 2023were retrospectively analyzed,and the relevant literature was discussed.Results:Among the 6 patients,5were female and 1was male,with a median age of 49(37~58)years old.All 6patients received BD chemotherapy for at least 2cycles,and all 6patients were mobilized with cyclophosphamide combined with granulocyte colony stimulating factor,and the median number of mononuclear cells was 3.5×10^(8)/kg and the m edian number of CD34+cells was 3.6×10^(6)/kg.After high-dose melphalan preconditioning,auto-HSCT was performed.All patients had good tolerance during chemotherapy and transplantation,with a median time of g ranulocyte implantation+10.5d,a median time of platelet implantation+12.5d,and no transplantation-related death.The median follow-up time was 34(16~57)months,and the overall survival rate was 83%(5/6).C onclusion:After 6patients with POEMS syndrome underwent the BD regimen followed by auto-HSCT,the normalization of the serum vascular endothelial growth factor(VEGF)level,the complete response of VEGF.The BD regimen followed by auto-HSCT is a safe and effective option for the treatment of POEMS syndrome.
作者
柳洁
张启科
冯友繁
伏媛
魏小芳
张雯洁
张媛媛
张少华
张尚艺
李晨阳
LIU Jie;ZHANG Qike;FENG Youfan;FU Yuan;WEI Xiaofang;ZHANG Wenjie;ZHANG Yuanyuan;ZHANG Shaohua;ZHANG Shangyi;LI Chenyang(First School of Clinical Medical,Gansu University of Chinese Medicine,Lanzhou,730000,China;Department of Hematology,Gansu Provincial Hospital)
出处
《临床血液学杂志》
2025年第3期220-224,共5页
Journal of Clinical Hematology
基金
甘肃省自然科学基金(No:22JR5RA660)
兰州市卫生健康科技发展项目(No:2019-004)
甘肃省人民医院国家级科研项目培育计划一般项目(No:19SYPYB-13)。
