CONTROL OF SEVERE BLEEDING EPISODE IN CASE OF GLANZMANN’S THROMBASTHENIA REFRACTORY TO PLATELET TRANSFUSION THERAPY BY ADMINISTERING RECOMBINANT FACTOR VIIa

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

Abstract


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

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