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阵发性睡眠性血红蛋白尿症(Paroxy-ScmrImoctunaI.hemogIobinuria)简称P.N.H,是一种少见的慢性血管内溶血性疾病〔1〕,主要是由于红细胞膜的获得性缺陷所致,并与血浆因素特别是补体有密切关系。1882年先为德国学者所描述,1911年Marchiafava氏,1931年MichcIi氏进一步报告,故又名Marchiafava-MicheIi综合,
Paroxysmal nocturnal hemoglobinuria (Paroxy-ScmrImoctunaI.hemogIobinuria) referred to as PNH, is a rare chronic intravascular hemolytic disease 〔1〕, mainly due to acquired defects of the erythrocyte membrane caused by, and in particular with plasma factors It is closely related to complement. 1882 first described by German scholars, Marchiafava in 1911, Michiel’s further report in 1931, it is also known as Marchiafava-MicheIi synthesis,