Bjørge Line, Ernst Peter, Haram Kjell O
Department of Obstetrics and Gynecology, Haukeland Hospital, Bergen, Norway.
Acta Obstet Gynecol Scand. 2003 Dec;82(12):1067-71. doi: 10.1046/j.1600-0412.2003.00337.x.
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia in which a defect of glycophosphatidylinositol (GPI)-anchored proteins in the cell membrane of bone marrow stem cells leads to increased sensitivity of the red cells to complement, causing intravascular hemolysis and hemoglobinuria. Other clinical features of this disease are cytopenia and an increased frequency of thrombotic events. We report a case of a pregnant woman with PNH on high-dosage anticoagulation therapy, the follow-up during the pregnancy, the delivery and the postpartum period. The obstetric literature on women with PNH is reviewed, the maternal and fetal risks are evaluated and the management of pregnancies and deliveries in such patients are discussed. During the pregnancy our patient was hypertransfused and used anticoagulation treatment. A healthy child was delivered in week 37 by cesarean section because of premature rupture of the membranes, unsuccessful induction and intrauterine infection. Because of bleeding problems a hysterectomy also had to be performed. In the postpartum period the patient developed her second episode of a liver vein thrombosis. She recovered gradually and 18 months after the delivery her disease is now in a stable phase. The literature shows a high maternal morbidity and mortality among pregnant PNH patients. Fetal wastage and prematurity rate are also high. Pregnancy in patients with PNH represents a high-risk situation for both the mother and the child and should not be recommended. A pregnant PNH woman should be followed closely by both obstetricians and hematologists.
阵发性睡眠性血红蛋白尿（PNH）是一种获得性溶血性贫血，其中骨髓干细胞细胞膜上糖磷脂酰肌醇（GPI）锚定蛋白的缺陷导致红细胞对补体的敏感性增加，引起血管内溶血和血红蛋白尿。该疾病的其他临床特征是血细胞减少和血栓形成事件的发生率增加。我们报告了一例接受高剂量抗凝治疗的PNH孕妇病例，以及其孕期、分娩期和产后的随访情况。本文回顾了关于PNH女性的产科文献，评估了母婴风险，并讨论了此类患者妊娠和分娩的管理。在孕期，我们的患者接受了多次输血并使用了抗凝治疗。由于胎膜早破、引产失败和宫内感染，患者在孕37周时通过剖宫产分娩出一名健康婴儿。由于出血问题，患者还接受了子宫切除术。产后，患者发生了第二次肝静脉血栓形成。她逐渐康复，分娩18个月后，病情目前处于稳定期。文献显示，PNH孕妇的孕产妇发病率和死亡率很高。胎儿流产和早产率也很高。PNH患者妊娠对母亲和孩子来说都是高风险情况，不应予以推荐。患有PNH的孕妇应由产科医生和血液科医生密切随访。
