Pregnancy with Paroxysmal Nocturnal Hemoglobinuria: A Case Series with Review of the Literature

Saudi J Med Med Sci. 2021 May-Aug;9(2):178-189. doi: 10.4103/sjmms.sjmms_4_20. Epub 2021 Apr 29.

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired hematopoietic stem cell disorder, and eculizumab and ravulizumab are its two approved therapies. Only few case series/reports have reported the outcomes of pregnancies in patients with PNH despite the increased risk of thrombosis. Similarly, there is limited knowledge regarding the effect of the approved treatments on conception and pregnancy outcomes. Here, we report the first series of pregnancies in PNH patients from the Middle Eastern region from our tertiary care hospital. Ten pregnancies in four females after diagnosis with PNH were identified. In terms of PNH management, only eculizumab was used, as the safety of ravulizumab use in pregnancies has not yet been established. In the antepartum period, the patients had variable symptoms that ranged from mild symptoms including epistaxis, tea-colored urine and vaginal bleeding to life-threatening vessel thrombosis. Further, red blood cell and platelet transfusions were required because of bleeding and hemolysis in four pregnancies. The pregnancy outcomes varied, but based on these, the safety of eculizumab use during pregnancy remained inconclusive. The postpartum period was complicated in one case by portal vein thrombosis and was managed accordingly. In conclusion, pregnant females with PNH are at an increased risk for complications due to PNH, and thus experienced hematologists and obstetricians should be involved jointly in their care.

Keywords: Complications; paroxysmal nocturnal hemoglobinuria; pregnancy.