• Asim Javed
  • Muhammad Ayyub
  • S Abrar
  • M Mansoor
  • Badshah Khan
  • T Hussain


lanzmann’s thrombasthenia is an autosomal recessive inherited platelet function defect. Though,quantitatively normal, the aggregation ability of platelets is reduced leading to bleeding episodesrequiring transfusion of platelet concentrates. We describe a case of 13-year-old girl who hadrecurrent episodes of epistaxis since birth and was managed with multiple platelet concentratetransfusions and recently admitted with severe epistaxis refractory to platelet transfusion. At thisstage administration of recombinant activated factor VII (fVIIa) was considered, which wasinitially given at 90 µg/kg dose with little control of bleeding but subsequent second dose of 120µg/kg was administered with excellent response and immediate control of bleeding.Keywords: Glanzmann’s thrombasthenia, autosomal recessive, platelet disorder, recombinantfactor VIIa, epistaxis


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