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Semin Thromb Hemost 2023; 49(06): 580-591
DOI: 10.1055/s-0042-1757183
Review Article

Von Willebrand Factor Multimer Analysis and Classification: A Comprehensive Review and Updates

Abdulrahman Saadalla
1   Department of Pathology, University of Utah Health Sciences Center and ARUP Laboratories, Salt Lake City, Utah
,
Jansen Seheult
2   Division of Hematopathology, Special Coagulation Laboratory, Mayo Clinic, Rochester, Minnesota
,
Rajiv K. Pruthi
2   Division of Hematopathology, Special Coagulation Laboratory, Mayo Clinic, Rochester, Minnesota
,
Dong Chen
2   Division of Hematopathology, Special Coagulation Laboratory, Mayo Clinic, Rochester, Minnesota
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Abstract

Von Willebrand factor (VWF) is a multimeric glycoprotein with essential roles in primary hemostasis. Patients with von Willebrand disease (VWD), due to quantitative and/or qualitative defects of VWF usually experience mucocutaneous bleeding. Based on the laboratory results of VWF antigen, various VWF activities, factor VIII activity, and VWF multimer patterns, VWD can be categorized as type 1, 2, and 3 VWD. VWF multimer analysis by either manual or semi-automated electrophoresis and immunoblotting is a critical part of the laboratory testing to differentiate type 1, type 2 VWD, and subtypes of type 1 or 2 VWD. The multimer distribution patterns can also help to understand the underlying molecular mechanism of VWF synthesis, multimerization, and clearance defects in VWD. This review will cover VWF synthesis, multimerization, secretion, VWF multimer analysis, and VWF multimer interpretation of various types and subtypes of VWD.

Keywords

von Willebrand factor - von Willebrand disease - multimer

Note

We dedicate this manuscript to Dr. William L. Nichols for his tremendous contribution to the patient care, education and research in hemostasis and thrombosis. Dr. Nichols died at the age of 81 on September 7th, 2022. It was always his desire to advance our understanding of hemostasis by careful clinical observation and precise laboratory testing, and share his knowledge via education and publication. As a physician, a scientist, a mentor, and a person with great generosity and warm heart and spirit, he is deeply missed.




Publication History

Article published online:
29 September 2022

© 2022. Thieme. All rights reserved.

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