期刊
BLOOD REVIEWS
卷 16, 期 3, 页码 193-202出版社
CHURCHILL LIVINGSTONE
DOI: 10.1016/S0268-960X(02)00030-9
关键词
Glanzmann Thrombasthenia; alpha(IIb)beta(3) integrin; platelet alloirnmunisation
类别
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 alloimnmunisation. (C) 2002 Published by Elsevier Science Ltd.
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