Von Willebrand Disease (VWD)
Part 3: VWF Activity Assay

VWF:RCo is Inadequate for Detecting AVW Abnormality

June 2011

We studied 23 patients who received left ventricular assist device implantation and 1 patient with polycythemia vera and marked thrombocytosis. All patients had subtle losses of the highest molecular weight VWF multimers. By using the receiver operating characteristic curve analysis, we studied the sensitivity and specificity of VWF ristocetin cofactor activity over antigen ratio. The area under the curve (AUC) is 0.75. When a 0.7 is set as the cut-off, its sensitivity of detecting such acquired VWF abnormality is less than 50%.

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Detecting AVW Abnormality

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Jump to section:

• Introduction
• Objectives
• History of VWF Ristocetin Cofactor Activity (VWF:RCo) Assay
• Problems with VWF:RCo Assay
• Summary of External Quality Assessment of VWF:RCo
• von Willebrand Disease(VWD) Diagnostic Error Rates Due to Erroneous VWF:RCo Results⁵
• VWF:RCo/Ag Ratio is Insensitive for Aquired von Willebrand Abnormalities (AVWA)
• VWF:RCo is Inadequate for Detecting AVW Abnormality
• The New Automated VWF Activity by Latex-Immunoturbidity Assay (VWF:Lx)
• VWF Activity Assay by an A1 Domain Conformation-Sensitive Antibody⁶
• Performance Characteristics of VWF:Lx
• VWF:Lx and VWF:RCo Method Comparison
• VWF:Lx and VWF:RCo Bias Analysis
• Sample Categorization
• Comparison of VWF:Lx/Ag and VWF:RCo/Ag Ratios
• VWF:Lx and VWF:RCo Errors
• Severe Type 1 vs 2M⁷
• VWF:Lx and RCo Errors
• VWF:Lx/Ag is Sensitive to AVWA with Subtle Loss of Highest HMWM
• ROC Analysis of VWF:Lx/Ag and VWF:RCo/Ag for Detecting AVWA with Loss of HMWMs
• VWF:Lx/Ag vs VWF:RCo/Ag Ratios
• VWF Laboratory Testing Strategy
• Sensitivity and Specificity of the Screening Panel
• von Willebrand Disease Profile
• von Willebrand Disease Profile
• von Willebrand Disease Profile
• von Willebrand Disease Profile
• Limitations of VWF:Lx
• Summary
• References
• Questions?