Intensive menstrual bleeding successfully treated with recombinant factor VIIa in glanzmann thrombasthenia

Abstract

Glanzmann thrombasthenia (GT) is a rare inherited qualitative platelet disorder due to the deficiency or defect of platelet membrane glycoprotein (GP) IIb/IIIa complex. Symptoms include purpura, petechiae, bruising, gingival bleeding, epistaxis, and menorrhagia. Platelet transfusion is considered the standard therapy for securing hemostasis in patients with GT when local measures and antifibrinolytic agents are inadequate. However, repeated platelet transfusions may result in GP IIb/IIIa and/or human leukocyte antigen (HLA) immunization and development of platelet refractoriness. Recombinant factor VIIa (rFVIIa) has been introduced as therapeutic alternative and has been suggested to be effective. Recombinant factor VIIa is indicated in Europe for the treatment of GT refractory of platelet transfusion. In previous studies, rFVIIa has been used in the prophylactic treatment of bleeding in patients with GT undergoing pelvic surgery, cesarean section, and vaginal delivery. In this article, we present a case of intensive menstrual bleeding refractory to previous antifibrinolytic agents and platelet transfusions but which responded well to treatment with rFVIIa. To our knowledge, there is no study or reported case in the literature reporting successful use of rFVIIa in a patient with excessive menstrual bleeding due to GT. © The Author(s) 2011.