Relationship between ABO blood group and von Willebrand factor levels: from biology to clinical implications
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BioMed Central
Open Access
Review
Relationship between ABO blood group and von Willebrand factor levels: from biology to clinical implications Massimo Franchini*1, Franco Capra2, Giovanni Targher3, Martina Montagnana4 and Giuseppe Lippi4 Address: 1Servizio di Immunoematologia e Trasfusione – Centro Emofilia, Azienda Ospedaliera di Verona, Verona, Italy, 2Medicina Interna C, Dipartimento di Scienze Biomediche e Chirurgiche, Università di Verona, Verona, Italy, 3Sezione di Endocrinologia e Malattie del Metabolismo, Dipartimento di Scienze Biomediche e Chirurgiche, Università di Verona, Verona, Italy and 4Istituto di Chimica e Microscopia Clinica, Dipartimento di Scienze Biomediche e Morfologiche, Università di Verona, Verona, Italy Email: Massimo Franchini* - [email protected]; Franco Capra - [email protected]; Giovanni Targher - [email protected]; Martina Montagnana - [email protected]; Giuseppe Lippi - [email protected] * Corresponding author
Published: 25 September 2007 Thrombosis Journal 2007, 5:14
doi:10.1186/1477-9560-5-14
Received: 6 August 2007 Accepted: 25 September 2007
This article is available from: http://www.thrombosisjournal.com/content/5/1/14 © 2007 Franchini et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract Although a number of studies have demonstrated the influence of ABO blood group on plasma levels of von Willebrand factor (VWF), the nature of this association and its clinical importance is still largely unknown. In this review, the most recent advances in our understanding of the mechanisms by which ABO blood group determines plasma VWF levels and their clinical impact will be discussed.
Review Introduction Von Willebrand factor (VWF) is a large adhesive glycoprotein synthesized by endothelial cells and megakaryocytes that circulates in the plasma as a series of heterogeneous multimers [1-3].
VWF has two major functions in hemostasis. First, it is essential for platelet-subendothelium adhesion and platelet-to-platelet interactions as well as platelet aggregation in vessels in which rapid blood flow results in elevated shear stress. Second, VWF is the specific carrier of factor VIII (FVIII) in plasma and protects it from proteolytic degradation, prolonging its half-life in circulation and efficiently localizing it at the site of vascular injury [4]. While a deficiency of VWF is responsible for a hemorrhagic diathesis (von Willebrand disease, VWD) [5], there
are increasing evidences that elevated VWF levels represent an important thrombotic risk factor [6,7]. Besides the VWF gene (12p12), it is well established that other gene loci exert major quantitative effects on VWF plasma levels. The most important of these loci has been shown to be the ABO blood group locus on chromosome 9q34 [8,9]. The antigens of the A
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