von Willebrand Disease: A Concise Review and Update for the Practicing Physician
Authors:
- 5 December 2016
- journal article
- review article
- Published by SAGE Publications in Clinical and Applied Thrombosis/Hemostasis
- Vol. 23 (8) , 900-910
Abstract
Von Willebrand disease (vWD) is the most common inherited disorder of hemostasis and comprises a spectrum of heterogeneous subtypes. Significant advances have been made in understanding von Willebrand factor (vWF) gene mutations, resultant physiologic deficits in the vWF peptide, and their correlation to clinical presentation. Diagnostic tests for this disorder are complex, and interpretation requires a thorough understanding of the underlying pathophysiology by the practicing physician. The objective of this review is to summarize our current understanding of pathophysiology, laboratory investigations, and evolving treatment paradigm of vWD with the availability of recombinant von Willebrand factor.Keywords
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