Molecular basis of Glanzmann's Thrombasthenia and current strategies in treatment

✍ Scribed by S Bellucci; J Caen

• Book ID: 104322967
• Publisher: Elsevier Science
• Year: 2002
• Tongue: English
• Weight: 157 KB
• Volume: 16
• Category: Article
• ISSN: 0268-960X
• DOI: 10.1016/s0268-960x(02)00030-9

✦ Synopsis

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.