异基因造血干细胞移植治疗EVI1阳性阵发性睡眠性再生障碍性贫血-血红蛋白尿综合征一例临床分析  

One Case Analysis of Allogeneic Hematopoietic Stem Cell Transplantation in Treatment of EVI1 Positive Aplastic Anemia- Paroxysmal Nocturnal Hemoglobinuria Syndome

作　　者：韦中玲[1] 戴艳[1] 黄来全[1] 蒋艺枝[1] 黄东平[1] WEI Zhong - ling DAI Yan HUANG Lai - quan JIANG Yi - zhi HUANG Dong -ping(Department of Hematology, Yijishan Hospital, Wannan Medical College, Wuhu 241000, China Department of Hematology, Yijishan Hospital, Wannan Medical College, Wuhu 241000, China)

机构地区：[1]皖南医学院弋矶山医院血液内科,安徽省芜湖市241000

出　　处：《中国全科医学》2016年第30期3765-3768,共4页Chinese General Practice

基　　金：安徽省教育厅科研基金支持项目(KJ2016SD58)

摘　　要：阵发性睡眠性血红蛋白尿是造血干细胞良性克隆性疾病,但常出现输血依赖的反复发作性溶血、骨髓衰竭、血栓等并发症,部分可转变为阵发性睡眠性再生障碍性贫血-血红蛋白尿(AA-PNH)综合征,导致不良预后。本文对1例接受清髓性异基因造血干细胞移植治疗且伴有EVI1阳性AA-PNH综合征患者的临床资料进行分析并复习相关文献,认为异基因造血干细胞移植可以作为治疗年轻AA-PNH综合征患者的一线治疗方式。Paroxysmal nocturnal hemoglobinuria is a benign clonal disease of hematopoietic stem cells,but it is often combined with complications such as recurrent hemolysis depended on transfusion,bone marrow failure,thrombosis,some can be transformed into aplastic anemia- paroxysmal nocturnal hemoglobinuria syndrome（ AA- PNH） syndrome and lead to poor prognosis. The paper analyzes the clinical data of using myeloablative allogeneic hematopoietic stem cell transplantation in treatment of one patient with EVI1 positive AA- PNH syndrome, and reviews related literatures; the author believes that allogeneic hematopoietic stem cell transplantation can be used as a way of first- line treatment for young AA- PNH syndrome patients.

关 键 词：血红蛋白尿 阵发性 贫血 再生障碍性 异基因造血干细胞移植 清髓性预处理 

分 类 号：R556.64[医药卫生—血液循环系统疾病]