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Clin Appl Thromb Hemost. 2017 Nov;23(8):900-910. doi: 10.1177/1076029616675969. Epub 2016 Dec 5.

von Willebrand Disease: A Concise Review and Update for the Practicing Physician.

Author information

1
1 Little Rock, Arkansas, USA.
2
2 Division of hematology/oncology, Department of Internal Medicine, Vancouver Island Cancer Center, University of British Columbia, British Columbia Cancer Agency, Vancouver, British Columbia, Canada.
3
3 British Columbia Cancer Agency, Vancouver, British Columbia, Canada.

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:

VWD; diagnostic tests for vWD; laboratory testing of vWD; recombinant von Willebrand factor; von Willebrand disease; von Willebrand factor

PMID:
27920237
DOI:
10.1177/1076029616675969
[Indexed for MEDLINE]

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