The Turkish Journal of Pediatrics 2003 , Vol 45 , Num 1
Pyelolithotomy in a patient with Glanzmann thrombasthenia and antiglycoprotein IIb/IIIa antibodies: the shortest possible duration of treatment with recombinant activated factor VII and platelet transfusions

Division of Hematology/Oncology, İstanbul University İstanbul Faculty of Medicine, İstanbul, Turkey

Division of Nephrology, Department of Pediatrics, İstanbul University İstanbul Faculty of Medicine, İstanbul, Turkey

Department of Urology, İstanbul University İstanbul Faculty of Medicine, İstanbul, Turkey

Abstract

Transfusion of platelet concentrates remains the first-line therapy for Glanzmann thrombasthenia in case of bleeding or preparation for surgery. However, development of antibodies to platelet glycoprotein (Gp) IIb/IIIa complex or human leukocyte antigens (HLA) is frequent and the main cause of platelet refractoriness. Recombinant activated factor VII (rFVIIa) is a potent alternative for patients with Glanzmann thrombasthenia with anti-platelet antibodies.

We describe a case of Glanzmann thrombasthenia with alloantibodies to platelet Gp IIb/IIIa complex who underwent a successful pyelolithotomy operation under the coverage of recombinant activated factor VIIa and platelet transfusions.

Keywords : Glanzmann thrombasthenia alloimmunization hematuria rVIIa platelet surgery
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