Molecular basis of Glanzmannʹs Thrombasthenia and current strategies in treatment

Glanzmann Thrombasthenia, an exceptional inherited platelet disorder is characterized by a complete lack of platelet aggregation due to a defect in the αIIbβ3 complex or to a qualitative abnormality of this complex. Advances in molecular biology have permitted to precise the molecular abnormality on αIIb or β3 genes responsible for the disease and have also contributed to a better knowledge of normal platelet physiology. Hemorrhages are the main clinical problem. Current principles of therapeutic management are proposed, with special reference to the risk of platelet alloimmunisation.