阵发性睡眠性血红蛋自尿(PNH)是获得性红细胞膜缺陷疾病。因红细胞膜吸附过多补体而溶血,常于睡眠时发作。近已证实PNH为干细胞病。 PNH之异常红细胞共分三型:第一型对补体的敏感度与正常红细胞相似;第二型对补体的敏感度为正常红细胞的三至五倍;第三型对补体的敏感度则为正常红细胞之十五至二十五倍。目前认为异常红细胞的产生系骨髓损害所致。膜的异常不仅见 Paroxysmal nocturnal hemoglobin (PNH) is an acquired erythrocyte membrane defect. Due to erythrocyte membrane adsorption of excessive complement and hemolysis, often attack during sleep. PNH has recently been confirmed as a stem cell disease. PNH abnormal erythrocytes are divided into three types: the first type of complement sensitivity is similar to normal red blood cells; the second type of sensitivity to complement three to five times the normal erythrocytes; the third type of sensitivity to complement is normal Erythrocytes fifteen to twenty-five times. It is currently believed that the production of abnormal red blood cells caused by bone marrow damage. Membrane abnormalities not only see