title: Pregnancy and paroxysmal nocturnal hemoglobinuria.
abstract: A patient is described who developed symptoms of paroxysmal nocturnal hemoglobinuria (PNH) in her first pregnancy. This was uneventful except for a spontaneous preterm delivery. The second pregnancy was complicated by severe anemia and a hemolytic crisis with Budd-Chiari syndrome at 31 weeks' amenorrhoea. Delivery was again preterm and was the result of induced labour after premature rupture of membranes at 34 weeks. Literature shows a high maternal mortality among PNH patients (5.8%). The most common cause of death is liver vein thrombosis (Budd-Chiari syndrome). Fetal wastage (30%) and prematurity rate (16%) are also high. Recommendations for follow-up and therapy are given such as anticoagulation therapy, platelets and washed erythocytes transfusions, screening for Budd-Chiari syndrome and infections.
authors.affiliation: Department of Obstetrics and Gynecology, Academic Medical Center, Amsterdam, The Netherlands.
authors.forename: J
authors.initials: J
authors.lastname: Bais
authors.forename: M
authors.initials: M
authors.lastname: Pel
authors.forename: A
authors.initials: A
authors.lastname: von dem Borne
authors.forename: H
authors.initials: H
authors.lastname: van der Lelie
mesh_terms: D000328:Adult; D000925:Anticoagulants / Q000627:therapeutic use; D017707:Erythrocyte Transfusion; D005260:Female; D005322:Premature Rupture of Fetal Membranes / Q000209:etiology; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications* / Q000628:therapy; D006801:Humans; D007751:Labor, Induced; D007752:Obstetric Labor, Premature / Q000209:etiology; D017713:Platelet Transfusion; D011247:Pregnancy; D011248:Pregnancy Complications
chemical_list: D000925:Anticoagulants