Comparison of a new automated von Willebrand factor activity assay with an aggregation von Willebrand ristocetin cofactor activity assay for the diagnosis of von Willebrand disease.

von Willebrand disease (VWD) is caused by quantitative and/or qualitative defects of von Willebrand factor (VWF). The HemosIL von Willebrand Factor Activity assay, a new automated immunological test to measure VWF activity, was implemented on STAC and compared with the von Willebrand ristocetin cofactor activity (VWF:RCo) aggregation method. Imprecision and dilution studies were also performed. Within-run imprecision was 17.2% and between-run imprecision was 8.3% (coefficients of variation). Dilution studies showed a linearity between 12.5 and 100%. Passing and Bablok regression comparing the HemosIL von Willebrand Factor Activity assay and the aggregation method yielded a slope of 1.25 (95% confidence interval: 1.11-1.38) and intercept of -1.40 (95% confidence interval: -8.07 to 0.00). The correlation coefficient was 0.84 (95% confidence interval: 0.78-0.89). With a cut-off value of 50% for VWF activity, the assay has a sensitivity of 94.1% and a specificity of 92.8%, compared with the VWF:RCo aggregation assay with a cut-off value of 60% producing a sensitivity of 100.0% (specificity 87.6%). With a cut-off value of 60%, the HemosIL von Willebrand Factor Activity assay on STAC is a reliable assay for VWD. VWF:RCo or other functional testing is still required to confirm the diagnosis and for further classification of VWD.