title: Molecular basis of Glanzmann's Thrombasthenia and current strategies in treatment.
abstract: Glanzmann Thrombasthenia, an exceptional inherited platelet disorder is characterized by a complete lack of platelet aggregation due to a defect in the alpha(IIb)beta(3) complex or to a qualitative abnormality of this complex. Advances in molecular biology have permitted to precise the molecular abnormality on alpha(IIb) or beta(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.
authors.affiliation: Service d'Hématologie Biologique, Hôpital Lariboisière, 2, rue Ambroise Paré, 75010, Paris, France.
authors.forename: S
authors.initials: S
authors.lastname: Bellucci
authors.forename: J
authors.initials: J
authors.lastname: Caen
mesh_terms: D019468:Disease Management; D005260:Female; D006470:Hemorrhage / Q000209:etiology / Q000628:therapy; D049673:History, 20th Century; D049674:History, 21st Century; D006801:Humans; D008297:Male; D009154:Mutation; D019039:Platelet Glycoprotein GPIIb-IIIa Complex / Q000235:genetics; D013915:Thrombasthenia / Q000188:drug therapy* / Q000209:etiology* / Q000266:history
chemical_list: D019039:Platelet Glycoprotein GPIIb-IIIa Complex