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Review
. 2015 May;37 Suppl 1(Suppl 1):11-7.
doi: 10.1111/ijlh.12345.

Laboratory diagnosis of von Willebrand disease

J C Roberts  1 , V H Flood  2   3
Affiliations
Review

Laboratory diagnosis of von Willebrand disease

J C Roberts et al. Int J Lab Hematol. 2015 May.

Abstract

Von Willebrand disease (VWD) is considered the most common inherited bleeding disorder and may also be the most difficult to diagnose. Clinical symptoms of VWD include predominantly mild mucosal bleeding; surgical bleeding may occur with specific challenges and joint bleeding can occur in the most severe forms. A family history either of diagnosed VWD or of bleeding symptoms is typically present. Laboratory diagnosis requires a series of assays of von Willebrand factor (VWF) quantity and function, and factor VIII activity, with no single straightforward diagnostic test available to either confirm or exclude the diagnosis. Newer assays of VWF function are becoming more available and useful in determining the laboratory diagnosis of VWD.

Keywords: Von Willebrand disease; platelets; von Willebrand factor.

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Conflict of interest statement

CONFLICT OF INTEREST

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Flow diagram for diagnosis of VWD.
See this image and copyright information in PMC

References

    1. Fujimura Y, Titani K, Holland LZ, Russell SR, Roberts JR, Elder JH, Ruggeri ZM, Zimmerman TS. Von Willebrand factor. A reduced and alkylated 52/48-kDa fragment beginning at amino acid residue 449 contains the domain interacting with platelet glycoprotein ib. J Biol Chem. 1986;261:381–5. - PubMed
    1. Pareti FI, Niiya K, McPherson JM, Ruggeri ZM. Isolation and characterization of two domains of human von Willebrand factor that interact with fibrillar collagen types I and III. J Biol Chem. 1987;262:13835–41. - PubMed
    1. Foster PA, Fulcher CA, Marti T, Titani K, Zimmerman TS. A major factor VIII binding domain resides within the amino-terminal 272 amino acid residues of von Willebrand factor. J Biol Chem. 1987;262:8443–6. - PubMed
    1. Sadler JE, Budde U, Eikenboom JC, Favaloro EJ, Hill FG, Holmberg L, Ingerslev J, Lee CA, Lillicrap D, Mannucci PM, Mazurier C, Meyer D, Nichols WL, Nishino M, Peake IR, Rodeghiero F, Schneppenheim R, Ruggeri ZM, Srivastava A, Montgomery RR, Federici AB Working Party on von Willebrand Disease Classification. Update on the pathophysiology and classification of von Willebrand disease: a report of the subcommittee on von Willebrand factor. J Thromb Haemost. 2006;4:2103–14. - PubMed
    1. Castaman G, Tosetto A, Cappelletti A, Goodeve A, Federici AB, Batlle J, Meyer D, Goudemand J, Eikenboom JC, Schneppenheim R, Budde U, Ingerslev J, Lethagen S, Hill F, Peake IR, Rodeghiero F. Validation of a rapid test (VWF-LIA) for the quantitative determination of von Willebrand factor antigen in type 1 von Willebrand disease diagnosis within the european multicenter study MCMDM-1VWD. Thromb Res. 2010;126:227–31. - PubMed

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