Bernard-Soulier syndrome in pregnancy; a report of four pregnancies in one patient, and review of the literature

Bernard-Soulier Syndrome is a rare autosomal recessive bleeding disorder characterized by a normal or low platelets count, the presence of giant platelets, and a prolonged bleeding time. Only five pregnant patients with this disease have been previously reported. Most of the complications seem to occur in the intrapartum or postpartum period. In some patients the disease can go unrecognized until the third or fourth decade. Our patient had two uneventful pregnancies, one pregnancy with early postpartum hemorrhage, and another pregnancy with intrapartum and late postpartum bleeding. Although the optimum mode of delivery is not clear yet, the vaginal route should be considered unless otherwise obstetrically indicated. Management of active bleeding episodes is also debatable; it includes platelets transfusions, desmopressin (DDAVP), antifibrinolytic therapy, and ecbolic agents for postpartum hemorrhage.