Glanzmann's thrombasthenia in pregnancy: a case and review of the literature

Abstract

Glanzmann's thrombasthenia is a rare autosomal recessive bleeding disorder resulting from a deficiency of glycoprotein IIb-IIIa complex in platelets. The deficient complex normally mediates platelet aggregation by binding adhesive proteins, which form bridges between activated cells. Despite normal platelet counts, morphology, prothrombin, and activated thromboplastin times, Glanzmann's thrombasthenia is characterized by a prolonged bleeding time and a severe hemorrhagic mucocutaneous diasthesis. Pregnancy and delivery are rare in these patients and have been associated with a high risk of severe hemorrhage. We present an unusual case in which a primi-gravida patient with Glanzmann's thrombasthenia underwent an uneventful pregnancy and spontaneous vaginal delivery, following intrapartum intravenous administration of single-donor platelets. Subsequent late postpartum hemorrhage required intravenous transfusion of an additional unit of single-donor platelets. In addition, we review the literature pertaining to pregnancy and Glanzmann's thrombasthenia with an emphasis on intrapartum prophylactic management.