阵发性睡眠性血红蛋白尿(简称 PNH)是一种原因不明的慢性溶血性贫血,其特点是阵发性血管内溶血伴有血红蛋白血症及血红蛋白尿症,特别在睡眠时明显。过去一直认为本病是一种少见的疾病,随着医疗诊断技术的提高及对本病的重视,目前国内外报导已明显增加,但其病因和发病机制尚未完全阐明。近年 Paroxysmal nocturnal hemoglobinuria (PNH) is an unexplained chronic hemolytic anemia, which is characterized by paroxysmal intravascular hemolysis accompanied by hemoglobinemia and hemoglobinuria, especially during sleep. In the past, the disease was thought to be a rare disease. With the improvement of medical diagnostic techniques and the emphasis on this disease, the number of reports at home and abroad has obviously increased at present, but its etiology and pathogenesis have not yet been fully elucidated. in recent years