Division of Hematology and Rheumatology, Department of Internal Medicine, Kinki University Faculty of Medicine, Osaka-Sayama, Osaka, Japan.
Int J Hematol. 2013 Apr;97(4):491-7. doi: 10.1007/s12185-013-1302-3. Epub 2013 Mar 2.
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired stem cell disorder characterized by intravascular hemolysis and thrombosis. The most serious complication is thrombosis, the risk of which is augmented by the hyper-coagulable state that occurs during pregnancy; despite this risk, however, young female PNH patients often desire to have a baby. We recently experienced two successful deliveries in PNH patients, who were treated with anticoagulant therapy during their pregnancies. Meanwhile, given the potential benefit of eculizumab (Soliris), a humanized monoclonal antibody against C5, in reducing thrombosis and hemolysis, it represents a promising therapeutic option for the treatment of pregnant PNH patients in combination with, or in replacement of, anticoagulant therapy.
阵发性睡眠性血红蛋白尿症（PNH）是一种获得性干细胞疾病，其特征是血管内溶血和血栓形成。最严重的并发症是血栓形成，妊娠期间发生的高凝状态会增加这种风险；然而，尽管存在这种风险，年轻的女性 PNH 患者通常仍希望生育。我们最近在两名接受抗凝治疗的 PNH 患者中成功分娩。同时，鉴于针对 C5 的人源化单克隆抗体依库珠单抗（Soliris）在减少血栓形成和溶血方面的潜在益处，它代表了一种有前途的治疗选择，可与抗凝治疗联合或替代抗凝治疗用于治疗妊娠 PNH 患者。
