Research on the experience of pregnancy in patients with paroxysmal nocturnal hemoglobinuria (PNH) has been limited, but a team of researchers recently reported on a case series involving 10 pregnancies in 4 patients with PNH in the Middle Eastern region. Findings were published in the Saudi Journal of Medicine & Medical Sciences.
PNH is associated with an increased thrombosis risk, and pregnancy in patients with this condition can carry enhanced risks of mortality and morbidity to both the mother and the fetus. However, there has not been extensive research on PNH with pregnancy or appropriate management of PNH in this context, according to the researchers, and limited information regarding rates of pregnancy with PNH in Saudi Arabia or the Middle Eastern region.
Patients with PNH in this retrospective case series were seen at the King Faisal Specialist Hospital & Research Center in Riyadh, Saudi Arabia. There were 20 patients with PNH who were seen at this center during the period between 2013 and 2021, 8 of whom were females. Of these patients, 6 had been of reproductive age, but 2 had died after hematopoietic stem cell transplantation. Each of the remaining 4 patients had become pregnant following the PNH diagnosis, and these 4 patients experienced a total of 10 pregnancies by the time of this analysis.
For management of PNH, eculizumab was the only treatment used, given the lack of established safety information regarding the use of ravulizumab in pregnancy. During pregnancy, symptoms of PNH varied among these patients. Mild presentations included epistaxis, tea-colored urine, pain, and vaginal bleeding. More serious symptoms included bone marrow suppression and a postpartum case of thrombosis. In 4 of the pregnancies, transfusions of red blood cells and/or platelets were needed, owing to episodes of bleeding and hemolysis.
Pregnancy outcomes were variable, and based on results with these patients, as well as a review of existing literature, the researchers considered the safety of eculizumab in pregnancy to be inconclusive.
The research team concluded that pregnancy with PNH imparted risks of PNH complications, and because of possible complications, they considered pregnancy to not be recommended with PNH. The researchers also concluded that management of patients with PNH during pregnancy should include specialists in both hematology and obstetrics.
Al-Dosari YM, Al-Zahrani H, Al-Mohareb F, Hashmi S. Pregnancy with paroxysmal nocturnal hemoglobinuria: a case series with review of the literature. Saudi J Med Med Sci. 2021;9(2):178-189. doi:10.4103/sjmms.sjmms_4_20