In order to compare quantitative and qualitative abnormalities of von Willebrand factor (vWF) in mediating platelet adhesion and thrombus formation, we performed ex vivo perfusion studies with native blood from patients with various types of von Willebrand disease (vWD). We studied twenty-seven patients having either a quantitative (types I, III) or a qualitative (type II) abnormality of vWF. At high shear rate (2600 s-1) we found that the defect in platelet collagen interactions in type I vWD was at the level of thrombus formation whereas platelet adhesion was normal. In the patients with a qualitative abnormality of vWF (type II), platelet adhesion and thrombus formation were markedly decreased at high shear conditions, indicating that the interaction of normal vWF with the platelet glycoprotein Ib is an essential initial step in platelet collagen interactions
Ex vivo experimental thrombosis in variants of von Willebrand disease / E. Fressinaud, A.B. Federici, G. Castaman, P.M. Mannucci, D. Meyer. - In: COMPTES RENDUS DE L'ACADÉMIE DES SCIENCES. SÉRIE 3, SCIENCES DE LA VIE. - ISSN 0764-4469. - 316:10(1993 Oct), pp. 1260-1265.
Ex vivo experimental thrombosis in variants of von Willebrand disease
A.B. FedericiSecondo
;P.M. MannucciPenultimo
;
1993
Abstract
In order to compare quantitative and qualitative abnormalities of von Willebrand factor (vWF) in mediating platelet adhesion and thrombus formation, we performed ex vivo perfusion studies with native blood from patients with various types of von Willebrand disease (vWD). We studied twenty-seven patients having either a quantitative (types I, III) or a qualitative (type II) abnormality of vWF. At high shear rate (2600 s-1) we found that the defect in platelet collagen interactions in type I vWD was at the level of thrombus formation whereas platelet adhesion was normal. In the patients with a qualitative abnormality of vWF (type II), platelet adhesion and thrombus formation were markedly decreased at high shear conditions, indicating that the interaction of normal vWF with the platelet glycoprotein Ib is an essential initial step in platelet collagen interactionsPubblicazioni consigliate
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