Use of rFVIIa in 4 children with Glanzmann thrombasthenia.

Inherited deficiencies of platelet surface glycoproteins, such as Glanzmann thrombasthenia (GT), occasionally result in severe bleeding episodes. Platelet transfusion is considered standard therapy for securing hemostasis in subjects with GT when local measures and antifibrinolytic agents are inadequate. We describe 4 case studies which suggest that recombinant activated factor VII may be an effective alternative to platelet transfusion in preventing or controlling bleeding, including surgical bleeding, in patients with GT.