Successful use of recombinant factor VIIa in a patient with acquired Glanzmann thrombasthenia

NO ABSTRACT - Immune thrombocytopenic purpura (ITP) is caused by circulating antibodies that react with target antigens on the platelet membrane [1]. In very rare cases, ITP may be associated with acquired Glanzmann thrombasthenia (GT), a severe bleeding disorder [2,3]. While congenital GT is caused by inherited mutations in the genes encoding a IIb or b3 subunits [4], acquired GT results from naturally occurring auto-antibodies directed against the a IIb b 3 complex, inhibiting its function. Patients classically present with thrombocytopenia and defective platelet aggregation in response to all physiological stimuli. ...