阵发性睡眠性血红蛋白尿症病理机制研究进展  

Research progress on pathological mechanism of paroxysmal nocturnal hemoglobinuria

作　　者：付蓉[1] 李丽燕[1] FU Rong;LI Li-yan(Department of Hematology,Tianjin Medical University General Hospital,Tianjin 300052,China)

机构地区：[1]天津医科大学总医院,天津300052

出　　处：《中国实用内科杂志》2022年第7期529-534,共6页Chinese Journal of Practical Internal Medicine

基　　金：国家自然科学基金面上项目(81770110);国家自然科学基金青年项目(82000128)。

摘　　要：阵发性睡眠性血红蛋白尿症(PNH)是一种获得性造血干细胞克隆性疾病,其病变细胞X染色体PIG-A基因突变与PNH细胞克隆增殖及疾病进展密切相关。深入研究其病理机制,有效识别异常克隆以明确诊断,寻找新的治疗靶点是需要解决的问题。文章就PNH病理机制研究进展作一述评。Paroxysmal nocturnal hemoglobinuria(PNH) is an acquired clonal disorder of hematopoiesis due to the inactivation of PIG-A gene which is closely related to the proliferation of PNH cells and the progress of disease.In depth study of its pathological mechanism,effective identification of abnormal clones for clear diagnosis and search for new therapeutic targets are the problems to be solved.This paper reviews the research progress on the pathological mechanism of PNH.

关 键 词：阵发性睡眠性血红蛋白尿症 克隆增殖 骨髓衰竭 血栓 

分 类 号：R552[医药卫生—血液循环系统疾病]