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1: Blood Rev. 2002 Sep;16(3):193-202.Click here to read Links

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

Bellucci S, Caen J.

Service d'Hématologie Biologique, Hôpital Lariboisière, 2, rue Ambroise Paré, 75010, Paris, France.

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.

PMID: 12163005 [PubMed - indexed for MEDLINE]