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阵发性睡眠性血红蛋白尿(PNH)是一种少见的慢性干细胞疾病,常伴阵发性溶血。对于该病合并妊娠的预后,日前还没有完整的评价。作者自1981年以来在法国、比利时、加拿大等国进行了回顾性和前瞻性研究,发现23例女性患者合并妊娠,其中PNH19例,AA/PNH 4例,平均年龄34.7岁,有8例于妊娠期间确诊。PNH患者于妊娠期发生急性溶血6例,出血6例;劳动后或产后发生急性溶血5例,出血3例,败血症或严重感染2例,肺栓塞1例。约1/3PNH合并妊娠患者无严重并发症,孕妇无1例死亡。所发生的38例次妊娠中流产11次,中止妊娠6
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare chronic disease of the stem cells, often accompanied by paroxysmal hemolysis. For the prognosis of the disease with pregnancy, a few days ago there is no complete evaluation. The authors conducted retrospective and prospective studies in France, Belgium, Canada and other countries since 1981 and found that 23 cases of female patients with pregnancy, including 19 cases of PNH, 4 cases of AA / PNH, the average age of 34.7 years, 8 cases of pregnancy During diagnosis. There were 6 cases of acute hemolytic and 6 cases of hemorrhage during pregnancy in PNH. There were 5 cases of acute hemolysis after operation or in postpartum, 3 cases of bleeding, 2 cases of sepsis or severe infection and 1 case of pulmonary embolism. About 1 / 3PNH patients with pregnancy complicated by no serious complications, no one pregnant woman died. Abortion occurred in 38 cases of secondary pregnancy 11 times, termination of pregnancy 6