Course of pregnancy and birth in a patient with Bernard-Soulier syndrome – a case report

Abstract

Bernard-Soulier syndrome is a rare autosomal recessive bleeding disorder characterized by platelet dysfunction, the presence of giant platelets, and a prolonged bleeding time. Absent or reduced expression of the platelet glycoprotein (GP) Ib-IX receptor results in the Bernard-Soulier syndrome and is characterized by severe bleeding and macrothrombocytopenia. To our knowledge only ten pregnant patients with this disease have been previously reported. In some patients the pregnancy course was uneventful while in others post-partum hemorrhage and resulting hysterectomy was the most common complication. We present a primiparous female with Bernard-Soulier syndrome who had delayed post-partum hemorrhage and review the literature.