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Research ArticleClinical Practice

Laboratory Diagnosis of von Willebrand Disease

Larry J. Smith
American Society for Clinical Laboratory Science April 2017, 30 (2) 65-74; DOI: https://doi.org/10.29074/ascls.30.2.65
Larry J. Smith
Abbott Diagnostics Division – Hematology Business Unit, Santa Clara, CA
PhD, SH(ASCP)
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    Figure 1.

    The von Willebrand Factor protein showing various functional domains that have been mapped to regions of the cDNA. These show sites of interaction with FVIII, GPIb, GPIIb/IIIa, collagen, and heparin. Various genetic mutations have been mapped to specific regions of the cDNA as shown above, and mutations within these domains result in different clinical expressions of VWD. A mutation in the D’ domain results in type 2N VWD where there is a loss of the FVIII binding ability of VWF. A mutation in the D3 domain results in types 2A and 2N VWD where there is a loss in the platelet binding ability of VWF or the ability to bind to FVIII. A mutation in the A1 domain results in types 2B and 2M VWD where there is either a gain-of-function mutation in the VWF molecule or a loss of the platelet binding ability of VWF. A mutation in the A2 domain results in type 2A VWD where there is a loss of the platelet binding ability of VWF.

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    Figure 2.

    Function of VWF. VWF supports platelet adhesion to the damaged endothelial layer and forms a complex with FVIII in the circulation.

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    Figure 3.

    VWF multimers. Low-resolution gel (0.65% agarose) demonstrating VWF multimer distribution. Lane 1 shows normal plasma as a control. Lane 2 shows type 1 VWD with all multimers sizes present, but reduced concentration. Lane 3 shows type 2A VWD with a loss of the high and intermediate multimers. Lane 4 shows type 2B with a loss of only the large multimers. Lane 3 show type 3 VWD with no detectable multimers

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    Figure 4.

    Latex immunoturbidometric assay. Latex beads are coated with a monoclonal antibody that binds to VWF.

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    Figure 5.

    Ristocetin cofactor assay measures the ability of VWF in plasma to agglutinate platelets in the presence of Ristocetin. The slope of the aggregation pattern is measured and the activity of VWF is determined from a calibration curve. (From Chrono-log VW Cofactor assay software, Chrono-Log Corporation, Havertown, PA).

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    Table 1.

    Classification of von Willebrand Disease

    Type 1 – partial quantitative deficiency
    Type 2 – qualitative defect
           2A – loss of high in intermediate molecular weight multimers
           2B – loss of the high molecular weight multimers
           2M – normal multimers
           2N – normal multimers
    Type 3 – complete absence
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    Table 2.

    Laboratory assays for von Willebrand Disease Diagnosis

    Initial assays for VWD
    1. von Willebrand factor antigen (VWF:Ag)

    2. von Willebrand factor activity (VWF:Act)

      1. Ristocetin cofactor activity (VWF:RCo)

      2. Von Willebrand factor immuno-activity (VWF:Lx)

      3. Collagen binding assay (VWF:CBA)

    3. FVIII clotting activity assay (FVIII:C)

    Specialized assays for classification of VWD
    1. von Willebrand factor multimers (VWFM)

    2. Ristocetin-induced platelet aggregation (RIPA)

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    Table 3.

    Classification of VWD by screening assays

    TypeVWF:Activity 50-166 (IU/dL)*VWF:Antigen 50-249 (IU/dL)*FVIII 50-186 (IU/dL)*Activity:Antigen ratioVWF Multimers
    Type 1<50<50NL or D~1Uniformly D
    Type 2
    ADNL or DNL or D<0.5-0.7Lg and Int D
    BDNL or DNL or D<0.5-0.7Lg D
    MDNL or DNL or D<0.5-0.7NL
    NNLNLNL~1NL
    Type 3<3<3<10--UD
    • NL, normal; D, decreased; Lg, large; Int, intermediate; UD, undetected

    • Reference ranges adapted from Rodak's Hematology: Clinical Principles and Applications, 5th ed. Elsevier, St. Louis, MO.

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American Society for Clinical Laboratory Science: 30 (2)
American Society for Clinical Laboratory Science
Vol. 30, Issue 2
Spring 2017
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Laboratory Diagnosis of von Willebrand Disease
Larry J. Smith
American Society for Clinical Laboratory Science Apr 2017, 30 (2) 65-74; DOI: 10.29074/ascls.30.2.65

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Laboratory Diagnosis of von Willebrand Disease
Larry J. Smith
American Society for Clinical Laboratory Science Apr 2017, 30 (2) 65-74; DOI: 10.29074/ascls.30.2.65
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Keywords

  • von Willebrand disease
  • von Willebrand factor
  • ristocetin cofactor
  • von Willebrand factor activity
  • von Willebrand factor antigen
  • collagen binding assay
  • von Willebrand factor multimers

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