Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2015 Mar 26;125(13):2029-37.
doi: 10.1182/blood-2014-08-528398. Epub 2015 Feb 23.

Diagnostic approach to von Willebrand disease

Christopher Ng  1 , David G Motto  2 , Jorge Di Paola  1
Affiliations
Review

Diagnostic approach to von Willebrand disease

Christopher Ng et al. Blood. .
No abstract available

PubMed Disclaimer

Figures

Figure 1
Figure 1
VWF structure. (A) Graphic representation of the VWF monomer with its different domains and their binding ligands. Note that the D1 and D2 domains (propeptide) are cleaved upon released of VWF from the endothelial cell. (B) Depiction of the ability of VWF monomers to dimerize through the C terminus and of those dimers to multimerize via disulfide bonds at the D3 domain, reaching sizes >20 000 kDa.
Figure 2
Figure 2
Mechanisms for VWD type 2. Schematic representation of VWF and its main interactions with platelets and FVIII, and how these interactions, when affected, cause VWF functional deficits and subsequent clinical bleeding.
Figure 3
Figure 3
Diagnostic algorithm. Algorithm of the diagnostic approach to mucocutaneous bleeding with a high clinical suspicion for VWD. The initial diagnosis is based on VWF:Ag, VWF:RCo, and FVIII:C whereas different types are diagnosed based on specialized tests. Specific groupings are based on the underlying pathophysiology of different VWD types. RIPA, ristocetin induced platelet aggregation.
See this image and copyright information in PMC

References

    1. von Willebrand EA. Hereditar pseudohemofili. Finska Lakarsallskapets. 1926;67:7–112.
    1. Haberichter SL, Castaman G, Budde U, et al. Identification of type 1 von Willebrand disease patients with reduced von Willebrand factor survival by assay of the VWF propeptide in the European study: molecular and clinical markers for the diagnosis and management of type 1 VWD (MCMDM-1VWD). Blood. 2008;111(10):4979–4985. - PMC - PubMed
    1. Fowler WE, Fretto LJ, Hamilton KK, Erickson HP, McKee PA. Substructure of human von Willebrand factor. J Clin Invest. 1985;76(4):1491–1500. - PMC - PubMed
    1. Federici AB, Bader R, Pagani S, Colibretti ML, De Marco L, Mannucci PM. Binding of von Willebrand factor to glycoproteins Ib and IIb/IIIa complex: affinity is related to multimeric size. Br J Haematol. 1989;73(1):93–99. - PubMed
    1. Moake JL, Turner NA, Stathopoulos NA, Nolasco LH, Hellums JD. Involvement of large plasma von Willebrand factor (vWF) multimers and unusually large vWF forms derived from endothelial cells in shear stress-induced platelet aggregation. J Clin Invest. 1986;78(6):1456–1461. - PMC - PubMed

Publication types

MeSH terms

Substances