The use of recombinant factor VIla in a primigravida with Glanzmann's thrombasthenia during delivery.

Glanzmann's thrombasthenia is an inherited hemorrhagic disorder characterized by a severe reduction in, or absence of, platelet aggregation in response to multiple physiologic agonists due to qualitative or quantitative abnormalities of platelet glycoprotein IIb-IIIa. Glanzmann's thrombasthenia is characterized by potentially major mucocutaneous bleeding and prolonged bleeding time. Platelet counts, platelet morphology, prothrombin, and activated thromboplastin times are all within normal ranges in patients with Glanzmann's thrombasthenia. Pregnancy and delivery are rare in Glanzmann thrombasthenia patients and have been associated with immediate postpartum hemorrhage. We describe the peripartum management of a 31-year-old primipara with Glanzmann's thrombasthenia who underwent spontaneous vaginal delivery. Four units of single-donor platelets, two units of packed red blood cells, 36 microg/kg recombinant human coagulation Factor VIIa (rFVIIa) were given during peripartum management.