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
