Pregnancy outcome of women with Paroxysmal Nocturnal Hemoglobinuria
Objective. To evaluate maternal and perinatal outcomes in pregnant women affected by Paroxysmal Nocturnal Hemoglobinuria (PNH) treated with eculizumab with a case series and literature review.
Methods. This was a case series study with literature review. Clinical records of all consecutive pregnant women with PNH were included in the study. The systematic review was conducted using electronic databases from inception of each database through May 2021. No restrictions for language or geographic location were applied. All reports of women with PNH in pregnancy, treated with eculizumab, were included in the review. Reports of women not treated or treated with other drugs rather than eculizumab (e.g. low molecular weight heparin alone) were excluded from the study. Maternal and perinatal outcomes were evaluated.
Results. Fifteen studies, including 24 pregnancies with PNH, were included in the review. All included women received eculizumab during the pregnancy, of them 10 received the treatment for the entire length of their gestation. Gestational age at delivery was reported in 18 cases, with preterm birth at less than 37 weeks occurring in fifteen women (83%). 57.1% of the women delivered by cesarean delivery. The cases series added three new cases in the literature. Two cases were already on eculizumab before pregnancy, while in one case eculizumab therapy was initiated in the second trimester of pregnancy. In all the three cases, there were no thrombotic complications, maternal or neonatal deaths, or fetal structural abnormalities.
Conclusions. PNH in pregnant women may be associated with an increased risk of obstetric complications, such us cesarean delivery or preterm birth. Eculizumab appears to be safe and effective for managing PNH during pregnancy.