NovoSeven (recombinant factor VIIa) for the treatment of bleeding episodes and perioperative management in patients with Glanzmann's thrombasthenia.

Glanzmann's thrombasthenia is a rare inherited autosomal recessive bleeding disorder caused by qualitative or quantitative defects of the platelet membrane glycoprotein IIb/IIIa. The ensuing lack of platelet aggregation is frequently associated with mucocutaneous bleeding that may be variable in both frequency and intensity, ranging from minimal bruising to severe and life-threatening hemorrhages. A number of treatment modalities have been proposed to manage the bleeding episodes, which include local measures, antifibrinolytic agents, platelet transfusions and recombinant activated factor VII. The role of this bypassing hemostatic agent for treatment or prevention of bleeding episodes in Glanzmann's thrombasthenia patients is critically analyzed in this review.