Perinatal outcomes of paroxysmal nocturnal hemoglobinuria during pregnancy: cases report and literature review / 中华围产医学杂志

ABSTRACT

Objective:To explore the progression of paroxysmal nocturnal hemoglobinuria (PNH) during pregnancy and treatment for improving maternal and infant outcomes.Methods:Nine pregnant women with PNH were admitted to the Obstetrics Department of Peking University People's Hospital from September 2010 to September 2020. The clinical data of these patients were retrospectively collected and analyzed. Relevant literature was reviewed to summarize the progression, treatment, complications, perinatal outcomes, and follow-up of PNH during pregnancy. Descriptive methods were used for statistical analysis.Results:Among the nine patients, six were classic PNH, and three combined with bone marrow failure disease. Eight cases received blood transfusion/low-dose corticosteroids or combination therapy during pregnancy, and four of them were also received anticoagulants. In seven out of the eight patients diagnosed prenatally, the disease worsened during pregnancy. Complications were noted in eight patients during pregnancy, including fetal growth restriction in seven cases, hypertension and premature delivery in four cases each, thrombosis and intrauterine fetal death in one case each. No maternal deaths were reported, with a live birth rate of 8/9 between 33-38 gestational weeks, with the median at 37 weeks. The median time of postpartum follow-up was 50 months (4-92 months), during which the patients' conditions were all stable, and no abnormalities were found in the growth and development of the babies.Conclusions:Pregnant women with PNH tend to worsen with an increased incidence of perinatal complications and adverse outcomes. Multidisciplinary management is recommended for this population may help improve maternal and infant outcomes.