Molecular basis of Glanzmann's 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 α IIb β 3 complex or to a qualitative abnormality of this complex. Advances in molecular biology have permitted to precise the molecular abnormality on α IIb or β 3 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.