Inherited platelet disorders: thrombocytopenias and thrombocytopathies
Dátum: 13.03.2010 13:05
Vec: Trombocytopenia
Inherited platelet disorders: thrombocytopenias and thrombocytopathies
Giovanna D'Andrea1, Massimiliano Chetta1, Maurizio Margaglione1,2
1Genetica Medica, Dipartimento di Scienze Biomediche, Università di Foggia;
2Unità di Emostasi e Trombosi, I.R.C.C.S. "Casa Sollievo della S offerenza", S. Giovanni Rotondo, Italy
Blood Transfusion 2009; 7: 278-292
Introduction
Platelets play an important role in normal haemostasis halting blood flow immediately after injuries through four fundamental different mechanisms: adhesion, aggregation, secretion, and expression of procoagulant activity. First, in the presence of vascular damage, platelets adhere to the connective tissue and, particularly when the damage occurs in vessels with a low shear rate, to subendothelial collagen, fibronectin and laminin. On the other hand, when damage occurs in regions with a high shear rate, platelet adhesion requires the presence of subendothelial von Willebrand factor (VWF) and specific platelet receptors, such as the glycoprotein Ib/IX/V (GPIb/IX/V) complex.
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