Diagnosing von Willebrand Disease: A Large Reference Laboratory's Perspective

Dorothy M. Adcock; Melissa Bethel; Andre Valcour

doi:10.1055/s-2006-947860

Seminars in Thrombosis and Hemostasis, Table of Contents

Semin Thromb Hemost 2006; 32(5): 472-479
DOI: 10.1055/s-2006-947860

Diagnosing von Willebrand Disease: A Large Reference Laboratory's Perspective

Dorothy M. Adcock¹ , Melissa Bethel¹ , Andre Valcour²

¹Esoterix, Inc., Aurora, Colorado
²Center for Esoteric Testing, Laboratory Corporation of America, Burlington, North Carolina

Abstract

ABSTRACT

Von Willebrand disease (vWD) is a common bleeding disorder. Diagnosis requires the demonstration of both clinical and laboratory features consistent with this disorder. Laboratory evaluation is complex, due in part to the variety of assays available and differing opinions regarding the optimum testing methodologies and algorithms required. This study represents a retrospective analysis of cases (n = 497) evaluated in our laboratory in which results for von Willebrand factor (vWF) multimeric analysis, vWF antigen, ristocetin cofactor activity, and collagen-binding activity were available. Results were compared to determine which assay parameters best correlate with one another, alone or in combination, and best correlate with the pattern and distribution of multimers. We demonstrate that performance of vWF activity or antigen assays in isolation can lead to both the overdiagnosis and underdiagnosis of vWD. Incorporation of the collagen-binding assay into the diagnostic algorithm leads to a reduced potential for misdiagnosis or misclassification of vWD and can reduce the number of multimeric analyses required dramatically.

KEYWORDS

von Willebrand disease (vWD) - collagen-binding assay - ristocetin cofactor assay - von Willebrand factor (vWF) - vWF multimers

Full Text

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Dorothy M AdcockM.D.

Esoterix, Inc.

3176 S. Peoria Ct, Aurora, CO 80014

Email: Dot.Adcock@esoterix.com