中国实用内科杂志2012，Vol.32Issue(5)：327-330,4.
阵发性睡眠性血红蛋白尿症现代诊断与治疗
The modern diagnosis and treatment of paroxysmal nocturnal hemoglobinuria
摘要
Abstract
Paroxysmal nocturnal hemoglobinuria, an acquired clonal hematopoietic stem cell disease, is X-linked and caused by PIG-A mutation. The clinical manifestations of PNH are mainly intravaseular hemolysis, bone marrow failure of different degrees and thrombosis. Since the abnormal clone exists with normal hematopoiesis in PNH patients, the pathological mechanism researches and diagnosis of this disease have been severely restricted. In recent years,the diagnosis of PNH has achieved some breakthroughs arising from the developments of various techniques,especially the applications of flow cy-tometry (FCM) and molecular biology approaches. There is a certain efficacy of corticosteroids-represented conventional treatment approaches for part of patients with primary PNH. The application of recombinant human anti-complement C5 monoclonal antibody,chemotherapy and stem cell transplantation have brought hopes for the steroid-ineffeclive or refractory and relapse PNH patients.关键词
阵发性睡眠性血红蛋白尿症/骨髓衰竭/血管内溶血/肾上腺皮质激素Key words
paroxysmal nocturnal hemoglobinuria/bone marrow failure/intravaseular hemolysis/glucocorticoid分类
医药卫生引用本文复制引用
付蓉..阵发性睡眠性血红蛋白尿症现代诊断与治疗[J].中国实用内科杂志,2012,32(5):327-330,4.
