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Pregnancy and paroxysmal nocturnal hemoglobinuria

J Bais1, M Pel, A von dem Borne

  • 1Department of Obstetrics and Gynecology, Academic Medical Center, Amsterdam, The Netherlands.

European Journal of Obstetrics, Gynecology, and Reproductive Biology
|March 15, 1994
PubMed

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.

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