Evaluation of the Abnormal Platelet Function in von Willebrand Disease by the Blood Filtration Test

Francesco I Pareti; Marco Cattaneo; Luca Carpinelli; Maddalena L Zighetti; Caterina Bressi; Pier Mannuccio Mannucci; Zaverio M Ruggeri

doi:10.1055/s-0038-1650600

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 1996; 76(03): 460-468
DOI: 10.1055/s-0038-1650600

Original Article

Schattauer GmbH Stuttgart

Evaluation of the Abnormal Platelet Function in von Willebrand Disease by the Blood Filtration Test

Francesco I Pareti

¹The Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Institute of Internal Medicine, IRCCS Maggiore Hospital and University of Milan, Italy

,

Marco Cattaneo

¹The Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Institute of Internal Medicine, IRCCS Maggiore Hospital and University of Milan, Italy

,

Luca Carpinelli

¹The Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Institute of Internal Medicine, IRCCS Maggiore Hospital and University of Milan, Italy

,

Maddalena L Zighetti

¹The Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Institute of Internal Medicine, IRCCS Maggiore Hospital and University of Milan, Italy

,

Caterina Bressi

¹The Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Institute of Internal Medicine, IRCCS Maggiore Hospital and University of Milan, Italy

,

Pier Mannuccio Mannucci

¹The Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Institute of Internal Medicine, IRCCS Maggiore Hospital and University of Milan, Italy

,

Zaverio M Ruggeri

²The Roon Research Center for Arteriosclerosis and Thrombosis, Division of Experimental Hemostasis and Thrombosis, Departments of Molecular and Experimental Medicine and of Vascular Biology, The Scripps Research Institute, La Jolla, CA, USA

› Author Affiliations

Abstract

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Summary

We have evaluated platelet function in different subtypes of von Willebrand disease (vWD) by pushing blood through the capillarysized channels of a glass filter. Patients, including those with type IIB vWD, showed lower than normal platelet retention and increased cumulative number of blood drops passing through the filter as a function of time. In contrast, shear-induced platelet aggregation, measured in the cone-and-plate viscometer, was paradoxically increased in type IIB patients. Treatment with l-desamino-8-D-arginine vasopressin (DDAVP) tended to normalize the filter test in patients with type I-platelet normal and type I-platelet low vWD, but infusion of a factor VUI/von Willebrand factor (vWF) concentrate lacking the largest vWF multimers was without effect in type 3 patients. Experiments with specific monoclonal antibodies demonstrated that the A1 and A3 domains of vWF, as well as the glycoproteins Ibα and Ilb-IIIa on platelets, are required for platelet retention in the filter. Thus, the test may reflect vWF function with regard to both platelet adhesion and aggregation under high shear stress, and provide relevant information on mechanisms involved in primary hemostasis.

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References

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