Chen Mann-Ling, Yu Chen-Hsiang, Chang Fong-Ming, Kuo Pao-Lin
Department of Obstetrics and Gynecology, National Cheng Kung University Medical Center, Tainan, Taiwan.
Taiwan J Obstet Gynecol. 2006 Sep;45(3):276-8. doi: 10.1016/S1028-4559(09)60242-4.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder characterized by complement-mediated intravascular hemolysis. As maternal complication of PNH is already severe, it becomes much more complex when preeclampsia is superimposed. We present a case of PNH superimposed with severe preeclampsia in the third trimester.
A 30-year-old, gravida 1, para 0, woman had PNH, diagnosed at the age of 17. Her PNH was stable under medication. In 2004, she conceived and had prenatal care at our hospital. At 35 weeks of gestation, preeclampsia with elevated blood pressure and proteinuria were superimposed and managed with close surveillance. A live male baby was delivered vaginally at 38 weeks of gestation. During parturition, her blood pressure increased to 180/100 mmHg. Thrombocytopenia, hyponatremia, hyperkalemia, hypoalbuminemia, elevated liver enzymes and lactate dehydrogenase were also noted. Preeclampsia continued to postpartum and eventually disappeared.
The most frequent causes of PNH-related fetomaternal morbidity and mortality are hemolysis and thrombosis. The situation becomes even more complicated when PNH is superimposed with preeclampsia. Appropriate clinical surveillance, awareness of the potential risks of hemolysis and thrombosis, as well as evaluation of fetal wellbeing are essential.
阵发性睡眠性血红蛋白尿（PNH）是一种罕见的血液系统疾病，其特征为补体介导的血管内溶血。由于PNH的孕产妇并发症已经很严重，当子痫前期与之叠加时，情况会变得更加复杂。我们报告一例孕晚期PNH合并重度子痫前期的病例。
一名30岁初产妇，0次分娩史，17岁时被诊断为PNH。其PNH在药物治疗下病情稳定。2004年，她怀孕并在我院接受产前检查。妊娠35周时，出现血压升高和蛋白尿的子痫前期，通过密切监测进行处理。妊娠38周时经阴道分娩一活男婴。分娩期间，她的血压升至180/100 mmHg。还发现有血小板减少、低钠血症、高钾血症、低白蛋白血症、肝酶和乳酸脱氢酶升高。子痫前期持续至产后，最终消失。
PNH相关的母胎发病和死亡的最常见原因是溶血和血栓形成。当PNH与子痫前期叠加时，情况会变得更加复杂。适当的临床监测、对溶血和血栓形成潜在风险的认识以及对胎儿健康的评估至关重要。
