Analysis of College of American Pathologists von Willebrand Factor Proficiency Testing Program

Eric Salazar; Thomas A. Long; Kristi Johnson Smock; Geoffrey D. Wool; Marian Rollins-Raval; Dong Chen; Neil Selwyn Harris; Clarence W. Chan; John D. Olson; Huy P. Pham; Jacob Ritter; David Unold; Amanda Matzke VanSandt; Andrew Jackson Goodwin IV

doi:10.1055/s-0042-1749591

Seminars in Thrombosis and Hemostasis, Table of Contents

Semin Thromb Hemost 2022; 48(06): 690-699
DOI: 10.1055/s-0042-1749591

Review Article

Analysis of College of American Pathologists von Willebrand Factor Proficiency Testing Program

Eric Salazar

¹Department of Pathology and Laboratory Medicine, UT Health San Antonio, San Antonio, Texas

,

Thomas A. Long

²Department of Biostatistics, College of American Pathologists, Northfield, Illinois

,

Kristi Johnson Smock

³Department of Pathology and ARUP Laboratories, University of Utah, Salt Lake City, Utah

,

Geoffrey D. Wool

⁴Department of Pathology, University of Chicago, Chicago, Illinois

,

Marian Rollins-Raval

⁵Department of Pathology, University of New Mexico, Albuquerque, New Mexico

,

Dong Chen

⁶Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota

,

Neil Selwyn Harris

⁷Department of Pathology, University of Florida, Gainesville, Florida

,

Clarence W. Chan

⁸Department of Pathology, University of Chicago Medical Center, Chicago, Illinois

,

John D. Olson

¹Department of Pathology and Laboratory Medicine, UT Health San Antonio, San Antonio, Texas

,

Huy P. Pham

⁹Seattle Apheresis Collection Center, National Marrow Donor Program (NMDP)Seattle, Washington

,

Jacob Ritter

¹Department of Pathology and Laboratory Medicine, UT Health San Antonio, San Antonio, Texas

,

David Unold

¹⁰Department of Pathology and Laboratory Medicine, University of California Davis Medical Center, Sacramento, California

,

Amanda Matzke VanSandt

¹¹Department of Pathology and Laboratory Medicine, Oregon Health & Science University, Portland, Oregon

,

Andrew Jackson Goodwin IV

¹²Department of Pathology, University of Vermont Medical Center, Burlington, Vermont

› Author Affiliations

Abstract

Von Willebrand factor (VWF) level and/or function is altered in von Willebrand disease (VWD), the most common heritable bleeding disorder worldwide. Laboratory assessment of VWF is continually evolving. Historically, the primary method for the assessment of VWF platelet-binding activity was the ristocetin cofactor assay (VWF:RCo). Contemporary alternative measures of VWF platelet-binding activity include VWF:GPIbR (recombinant; using ristocetin), VWF:GPIbM (recombinant; gain-of-function mutant), and monoclonal antibody. Recently, the American Society of Hematology, International Society on Thrombosis and Haemostasis, National Hemophilia Foundation, and World Federation of Hemophilia collaboration issued guidelines recommending the use of newer assays of VWF platelet-binding activity (VWF: GPIbM, VWF: GPIbR) over VWF:RCo, given known limitations of the VWF:RCo assay. Despite this recommendation, the newer VWF:GPIbM and VWF:GPIbR assays are not United States Food and Drug Administration cleared, limiting their availability in the United States. We sought to assess assay utilization trends, agreement of VWF testing methods, and imprecision of VWF testing (based on assigned sample type) from the College of American Pathologists Proficiency Testing Surveys. The analysis confirms that, while VWF antigen testing has low imprecision, the various VWF activity assays have significant interassay variability, with VWF:RCo showing greater imprecision than the newer GPIb-binding assays. The overall trends in assay utilization reflect the barriers to complete compliance with modern VWD diagnostic guidelines in North America.

Keywords

von Willebrand disease - coagulation special testing - proficiency testing

Full Text

References

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