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Blood Rev. 2002 Sep;16(3):193-202.

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

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  • 1Service d'Hématologie Biologique, Hôpital Lariboisière, 2, rue Ambroise Paré, 75010, Paris, France.

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.

PMID:
12163005
[PubMed - indexed for MEDLINE]
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