阵发性睡眠性血红蛋白尿症诊治现状与展望  

Advancements in Diagnosis and Management of Paroxysmal Nocturnal Hemoglobinuria——Review

作　　者：孙莺心[1] 何广胜[1] 

机构地区：[1]苏州大学附属第一医院 江苏省血液研究所 卫生部血栓与止血重点实验室,江苏苏州215006

出　　处：《中国实验血液学杂志》2012年第5期1267-1271,共5页Journal of Experimental Hematology

基　　金：国家科技支撑计划(编号2008BAI61B02);国家科技重大专项课题(编号20082X09312-026);江苏高校优势学科建设工程资助项目;江苏省医学重点人才(编号H201126)项目资助;高校自然科学研究项目(编号09KJB320015)

摘　　要：阵发性睡眠性血红蛋白尿症(PNH)是一种补体异常活化的溶血性疾病,嗜水气单胞菌毒素变异体(FLAER)检测法提高了诊断的敏感性和特异性,为PNH的早发现、早治疗打下了良好的基础。血栓形成、肺动脉高压、慢性肾功能不全等非典型症状日益受到关注。Eculizumab极大地改善了PNH的治疗现状。异基因造血干细胞移植仍为其惟一治愈的手段。本文就PNH的FLAER诊断,临床症状和治疗研究进展进行综述。Paroxysmal nocturnal hemoglobinuria（PNH） is a hemolytic disease of abnormally activated complement.FLAER diagnosis is a higher sensitive and specific method,which makes PNH patients to be early discovered and treated.Non-typical symptoms inclucling thrombosis,pulmonary hypertension and chronic kidney disease in PNH have been caused increasing attention.Eculizumab monocloual antibody has greatly improved the current treatment status of PNH.PNH can be cured thoroughly by allogeneic hematopoietic stem cell transplantation.In this article,the FLAER diagnosis,clinic symptoms and progress of treatment in patients with PNH are reviewed.

关 键 词：阵发性血红蛋白尿 FLAER诊断 ECULIZUMAB 

分 类 号：R556.64[医药卫生—血液循环系统疾病]