Abstract • 149

Introduction. Glanzmann thrombasthenia is a rare autosomal recessive inherited bleeding disorder caused by the deficiency of platelet membrane glycoproteins IIb/IIIa resulting in platelet dysfunction. Platelet transfusion is usually used for treatment of severe bleeding and for surgical prophylaxis. Platelet transfusions may result in the development of HLA or platelet antibodies rendering platelet transfusion ineffective, and may carry the risk of adverse reactions including the transmission of human viruses. rFVIIa, a recombinant product effective in the management of hemophilia with factor VIII or IX inhibitors, has recently been reported to be effective in treating bleeding episodes in a few patients with platelet bleeding disorders.

Patient Report. This is a 5 year old boy with Glanzmann thrombasthenia who previously had received several platelet transfusions for severe epistaxis. He developed left inguinal hernia and underwent left hernia repair and right inguinal exploration. rFVIIa 99 µg/kg was given just prior to surgical incision, and continued at 2 hourly intervals for 24 hours (total 13 doses). EACA (Amicar™) was given for 10 days. There was no abnormal bleeding intra- or post-operatively. Only minor wound ecchymoses were observed.

Conclusions. rFVIIa, given as a short course, together with antifibrinolytic agent, were effective for surgical prophylaxis in this patient with Glanzmann thrombasthenia. We need additional assessment of the safety and efficacy of rFVIIa as an alternative to platelet transfusion in platelet bleeding disorders.