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CASE SERIES
Year : 2021  |  Volume : 9  |  Issue : 2  |  Page : 178-189

Pregnancy with paroxysmal nocturnal hemoglobinuria: A case series with review of the literature


1 Internal Medicine Department, Bahrain Defence Force Hospital and Royal Medical Services; King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
2 Adult Hematology/Bone Marrow Transplantation Section, Oncology Center, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
3 Adult Hematology, HSCT Section, Oncology Center, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
4 Adult Hematology/Bone Marrow Transplantation Section, Oncology Center, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia; Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA

Correspondence Address:
Yara Mohammad Al-Dosari
King Faisal Specialist Hospital and Research Center, PO Box 3354, Riyadh 11211
Saudi Arabia
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DOI: 10.4103/sjmms.sjmms_4_20

PMID: 34084110

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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.


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