PNH是中国北方最常见的溶血病之一。临床及血液学表现不一,病情轻重各异,易致误诊。除常用诊断方法外,经我们简化的蛇毒因子溶血试验可在78％病人中呈阳性,假阳性者罕见。截至1984年我院诊断的128例中,14例由再障转化而来,46例从无血红蛋白尿发作,6例有血管栓塞,1例有胆石症,2例曾有急性肾功能衰竭。在70例随诊5年以上或已知转归者中,5例自然痊愈,1例死于急性白血病。本病 PNH is one of the most common hemolytic diseases in northern China. Clinical and hematological manifestations vary in severity, prone to misdiagnosis. In addition to the common diagnostic methods, our simplified venom hemolysis test is positive in 78% of patients and is rare in false-positives. Of the 128 cases diagnosed in our hospital in 1984, 14 were converted from aplastic anemia, 46 from hemoglobinuria, 6 with vascular embolism, 1 with cholelithiasis, and 2 with acute renal failure. Of the 70 patients who were followed up for more than 5 years or those who were known, 5 were cured naturally and 1 died of acute leukemia. The disease