【摘要】 对２０３例门诊及住院确诊为阵发性睡眠性血红蛋白尿症（ＰＮＨ）的病人（按１９８７年全国会议诊断标准），进行回顾性分析。目的在于总结经验，探讨延误诊断的原因。结果表明：首发症状为贫血（５６．７％），次为出血（２４．６％），血红蛋白尿仅占１２．８％。常用的Ｈａｍ试验（酸化血清溶血）在１０．４％病人中始终阴性。因此，一年内确诊者仅３５．５％，首次诊断常为再生障碍性贫血或不明原因贫血。延误诊断的因素为临床表现不典型、隐匿发病、缺乏敏感及特异性强的试验诊断方法，但最主要的是医生忽略本病的可能性。经验教训是在鉴别诊断时要时时想到本病；不能因一次Ｈａｍ试验为阴性而否定本病，常需多项多次检查，一时难于诊断者需长期密切追查。更多还原

【Abstract】 wo hundred and three cases of PNH diagnosed(according to the National Unified Diagnosed Criteri-a,1987) and treated from 1949 to 1992 1n Peking Union MedicaI Hospital were analyzed retrospectively in an attempt to investigate the factors leading to delayed diagnosis. The presenting manifestation was anemia in 56.7%of the patients. Next was hemorrhage(24.6%).Hemoglobinuria was seen only in 12.8%of the cases at the onset. Ham test was persistently negative in 10.4% of the patients. Only one third of all the cases was correctly diagnosed as PNH within one year after onset.Aplastic anemia or anemia of un-known cause was first diagnosed in 60.1%of the cases,The heterogeneity of the manifestations and the lack of sensitive and also specific laboratory tests were the factors leading to delayed diagnosis,but negli-gence of PNH in differential diagnosis seem to be a major the main cause,Constant awareness of the possi-bility and careful follow-up accompanied with repeated laboratory examinations in a suspected case are cru-cial for early diagnosis of PNH.更多还原