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VWFM2<br />

8844<br />

preferably as a panel of tests with reflexive testing and interpretive reporting. Within this context, von<br />

Willebrand factor antigen measurement can be useful for: -Diagnosis of von Willebrand disease and<br />

differentiation of von Willebrand disease subtype -Differentiation of von Willebrand disease from<br />

hemophilia A (in conjunction with factor VIII coagulant assay) Monitoring therapeutic efficacy of<br />

treatment with DDAVP (desmopressin) or von Willebrand factor concentrates in patients with von<br />

Willebrand disease<br />

Interpretation: von Willebrand factor (VWF) antigen assay results generally must be used together<br />

with assays of VWF ristocetin cofactor activity and factor VIII coagulant activity, for optimum clinical<br />

utility and diagnostic efficiency. The diagnosis of von Willebrand disease (VWD) requires a combination<br />

of clinical and laboratory information. We suggest ordering VWPR/83099 von Willebrand Profile.<br />

Patients with congenital severe type III VWD have a markedly decreased or undetectable level of VWF<br />

antigen in the plasma (and in the platelets), in addition to a plasma ristocetin cofactor activity that is very<br />

low or not detectable. Patients with types IIA and IIB variants of VWF (with abnormal plasma VWF<br />

function and multimeric structure) may have normal or decreased plasma VWF antigen. However, they<br />

typically have decreased plasma ristocetin cofactor activity, along with decreased higher<br />

molecular-weight VWF multimers in the plasma. Patients with types IIM or IIN VWD have normal levels<br />

of VWF antigen. In spite of this, they either have decreased vWF ristocetin cofactor activity, not caused<br />

by absence of higher molecular weight vWF multimers (type IIM VWD), or decreased factor VIII<br />

coagulant activity (type IIN VWD) Patients with type I VWD (with decreased but normally functioning<br />

plasma VWF) have concordantly decreased plasma VWF antigen and ristocetin cofactor activity. Patients<br />

with acquired VWD may have either normal or decreased plasma VWF antigen.<br />

Reference Values:<br />

55-200%<br />

Note: Individuals of blood group "O" may have lower plasma von Willebrand factor (VWF) antigen than<br />

those of other ABO blood groups, such that apparently normal individuals of blood group "O" may have<br />

plasma VWF antigen as low as 40% to 50%, whereas the lower limit of the reference range for individuals<br />

of other blood groups may be 60% to 70%.<br />

Children: Neonates, infants, and children have normal or mildly increased plasma VWF antigen, with<br />

respect to the adult reference range.<br />

Clinical References: 1. Sadler JE, Blinder M: von Willebrand disease: diagnosis, classification and<br />

treatment. In Hemostasis and Thrombosis: Basic Principles and Clinical Practice. 5th edition. Edited by<br />

RW Colman, VJ Marder, AW Clowes, et al. Baltimore, MD, Lippincott Williams & Wilkins, 2006, pp<br />

905-921 2. Eby C, Chance D, Oliver D: A multicenter evaluation of ATA-LIATEST VWF: A new latex<br />

particle immunoassay for von Willebrand factor antigen. Clin Hemostasis Rev 1997;11:16-17 3.<br />

Rodeghiero F, Castaman G, Tosetto A: Von Willebrand factor antigen is less sensitive then ristocetin<br />

cofactor for the diagnosis of type K von Willebrand disease – Results based on an epidemiological<br />

investigation. Thromb Haemost 1990;64:349-352 4. Triplett DA: Laboratory diagnosis of von<br />

Willebrandâ€s disease. <strong>Mayo</strong> Clin Proc 1991;66:832-840<br />

von Willebrand Factor Multimer Analysis, Plasma<br />

Clinical Information: von Willebrand factor (VWF) is a large multimeric plasma glycoprotein that<br />

performs 2 critical functions in hemostasis: -VWF is a ligand and mediates platelet adhesion to the<br />

subendothelial matrix at the site of vessel wall injury by binding to the constitutively active platelet<br />

receptor glycoprotein (GP)-Ib, V, IX complex, and to subendothelial matrix collagen -VWF is a carrier<br />

molecule for procoagulant factor VIII in the circulation, increasing the factor VIII half-life 5-fold. Under<br />

conditions of high shear, VWF also mediates platelet-platelet cohesion by binding to the platelet receptor<br />

GP-IIb/IIIa (integrin alpha IIb beta3) A bleeding disorder, von Willebrand disease (VWD), occurs when<br />

VWF is quantitatively deficient or qualitatively abnormal. VWD manifests clinically as easy bruising,<br />

mucocutaneous bleeding (eg, epistaxis, menorrhagia), and bleeding after trauma or surgery. VWD is the<br />

most common of the inherited bleeding disorders, and can also occur on an acquired basis. Plasma VWF<br />

consists of a series of multimers varying in size from dimers to multimers over 40 subunits (>10 million<br />

Daltons). The largest multimers provide multiple binding sites that can interact with both platelet<br />

receptors and subendothelial matrix sites of injury, and are the most hemostatically active form of VWF.<br />

Current as of January 4, 2013 7:15 pm CST 800-533-1710 or 507-266-5700 or <strong>Mayo</strong><strong>Medical</strong><strong>Laboratories</strong>.com Page 1863

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