Department of Hematology and Oncology, Tufts Medical Center, 800 Washington St, Boston, MA 02111, USA.
Leuk Res. 2010 May;34(5):566-71. doi: 10.1016/j.leukres.2009.10.025. Epub 2009 Dec 1.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal stem cell disorder, which affects women of child-bearing age. PNH is associated with thrombotic complications, which are the main causes of morbidity and mortality. Management of a pregnant woman with PNH remains a challenge due to high incidence of thrombotic complications and the difficulty of differentiating a PNH crisis from the complications of pregnancy. PNH is associated with an increased rate of premature labor and fetal loss. Eculizumab, a humanized monoclonal antibody directed against the terminal complement protein C5, has revolutionized treatment of PNH. However, the role of eculizumab in pregnancy is unclear. We review the current strategies for the management of pregnant women with PNH, underline the controversies and present our recommendations.
阵发性睡眠性血红蛋白尿症(PNH)是一种罕见的克隆性干细胞疾病,影响育龄妇女。PNH 与血栓并发症有关,这些并发症是发病率和死亡率的主要原因。由于血栓并发症的发生率高,以及区分 PNH 危象与妊娠并发症的难度,管理患有 PNH 的孕妇仍然是一个挑战。PNH 与早产和胎儿丢失的发生率增加有关。依库珠单抗,一种针对末端补体蛋白 C5 的人源化单克隆抗体,彻底改变了 PNH 的治疗方法。然而,依库珠单抗在妊娠中的作用尚不清楚。我们回顾了目前管理患有 PNH 的孕妇的策略,强调了争议并提出了我们的建议。