阵发性睡眠性血红蛋白尿(简称PNH)是一种罕见的获得性溶血性贫血。本病血循环中的红细胞对激活的血清补体异常敏感而发生溶解。顾名思义,红细胞过多破坏主要发生在夜间,因此仅仅(或主要)在清晨发现血红蛋白尿。血小板和粒细胞也对补体异常敏感,本病常有血小板或白细胞减少,或有两者同时减少。 Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hemolytic anemia. Erythrocytes in the blood circulation of the disease are very sensitive to activated serum complement and dissolve. As its name implies, the destruction of red blood cells occurs mainly at night, so hemoglobinuria is found only (or mainly) early in the morning. Platelets and granulocytes are also abnormally sensitive to complement, the disease often have thrombocytopenia or leukopenia, or both at the same time reduced.