摘要

Introduction: The effective diagnosis and monitoring of Von Willebrand Disease (VWD) requires an accurate assessment of ristocetin co-factor activity (VWF:RCo). Current methodologies include automated platelet aggregometry and manual visual agglutination both of which are laborious to perform and notoriously subject to a high degree of inter and intra assay variation.
Methods and Materials: We have evaluated an automated VWF: RCo assay (BC Von Willebrand Reagent, Siemens, Marberg, Germany) for use on the Sysmex CS2100i analyser (Milton Keynes, UK) and retrospectively compared the results with an in-house manual visual agglutination assay and VWF antigen (Siemens) in normal subjects and in 53 patients with various types of VWD and 23 patients following VWF therapeutic treatment.
Results: The intra and interassay CV was improved with the automated assay (2.3% and 3.8% respectively) compared to 7% with the manual VWF: RCo assay. Good correlation was found between the two assays (r = 0.91) in 53 patients with VWD. The mean manual VWF: RCo was 0.25 IU/ml and mean automated VWF: RCo was 0.27 IU/ml. A comparable increase in VWF: RCo following treatment, mostly with Desmopressin, was found in 13 patients with type 1 VWD (mean 3.9 fold increase with manual VWF: RCo and 3.1 fold with the automated VWF: RCo). In 13 patients with type 2 or 3 VWD following treatment mostly with concentrate, a higher increase was found with the automated VWF: RCo assay than the manual assay (mean 11.9 fold manually and mean 20.3 automated).
Conclusion: The automated VWF: RCo assay shows enhanced precision and analysis time in this difficult and time consuming laboratory test and its introduction should greatly improve the reliability of VWF testing.

  • 出版日期2011-4