A Successful Planned Pregnancy and Delivery with Eculizumab Maintenance in a Woman with Paroxysmal Nocturnal Hemoglobinuria

ABSTRACT

Paroxysmal nocturnal hemoglobinuria (PNH) is a hematologic disorder characterized by complement-mediated hemolysis leading to severe complications, such as life threatening thrombosis. Eculizumab, a humanized anti-C5 monoclonal antibody, has dramatically improved outcomes of patients with PNH. Despite this new revolutionary treatment, clinical information regarding eculizumab use in pregnant women with PNH is limited. A 30-year-old female with PNH underwent acute aggravation of PNH presented with acute kidney injury (AKI) triggered by an infectious event. After the stabilization of AKI with supportive care and later continuous eculizumab use, a planned pregnancy was attempted and achieved because she and her spouse wanted to have a baby. We monitored the patient carefully throughout her pregnancy with 100 mg/day of aspirin and the maintenance of 900 mg of intravenous eculizumab every 2 weeks. She remained stable during pregnancy and a successful delivery was achieved without maternofetal complication.