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Article Dans Une Revue Haemophilia Année : 2015

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

Résumé

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. ...

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Dates et versions

hal-02980612 , version 1 (27-10-2020)

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M. Tuffigo, E. Lazaro, C. James, C. Subtil, J. F. Viallard, et al.. Successful use of recombinant factor VIIa in a patient with acquired Glanzmann thrombasthenia. Haemophilia, inPress, 1 (1), pp.116-118. ⟨10.1111/hae.12589⟩. ⟨hal-02980612⟩

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