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阵发性睡眠性血红蛋白尿(简称 PNH)是一种少见的原因不明的慢性溶血性贫血。临床以发作性睡眠时加重的血红蛋白尿和全血细胞减少为特征,基本病变为获得性红细胞膜的缺陷,对补体溶血敏感。典型的病例一般诊断不难,但应与其它溶血性贫血鉴别,而无血红蛋白尿发作者诊断较为困难,易与再生障碍性贫血(再障)、缺铁性贫血、巨幼红细胞性贫血或肝胆疾病相混淆。我院自1972年10月到1979年10月共收治 PNH 28例,其中18例误诊。为吸取教训,提高对本病的认识,将18例误诊情况分析如下:18例中诊断为再障6例、缺铁性贫血4例、肝炎
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare cause of chronic hemolytic anemia. Clinically, episodes of sleep increased hemoglobinuria and pancytopenia is characterized by the basic lesion of acquired erythrocyte membrane defects, complement and hemolytic sensitive. Typical cases are not difficult to diagnose in general, but should be distinguished from other hemolytic anemias, whereas those without hemoglobinuria are more difficult to diagnose and are often associated with aplastic anemia (aplastic anemia), iron-deficiency anemia, megaloblastic anemia or hepatobiliary Confused disease. Our hospital from October 1972 to October 1979 were treated 28 cases of PNH, of which 18 cases were misdiagnosed. In order to learn the lesson and raise awareness of the disease, 18 cases of misdiagnosis were analyzed as follows: 18 cases were diagnosed as aplastic anemia in 6 cases, iron deficiency anemia in 4 cases, hepatitis