快速、自动化的 VWF 瑞斯托霉素辅因子活性测定可提高 Von Willebrand 病诊断的可靠性。

A rapid, automated VWF ristocetin cofactor activity assay improves reliability in the diagnosis of Von Willebrand disease.

机构信息

Sheffield Haemophilia and Thrombosis Centre, Sheffield, UK.

出版信息

Thromb Res. 2011 Apr;127(4):341-4. doi: 10.1016/j.thromres.2010.11.029. Epub 2010 Dec 24.

DOI:10.1016/j.thromres.2010.11.029

PMID:21186048

Abstract

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 0.25IU/ml and mean automated VWF:RCo was 0.27IU/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.

摘要

简介

有效的诊断和监测血管性血友病（VWD）需要准确评估瑞斯托霉素辅因子活性（VWF：RCo）。目前的方法包括自动化血小板聚集测定法和手动视觉凝集法，这两种方法都很繁琐，并且非常容易受到实验内和实验间变异性的影响。

方法和材料

我们评估了一种用于 Sysmex CS2100i 分析仪（英国米尔顿凯恩斯）的自动化 VWF：RCo 测定法（BC 血管性血友病试剂，西门子，德国马尔堡），并回顾性地将结果与内部手动视觉凝集测定法和 VWF 抗原（西门子）在正常受试者和 53 例各种类型 VWD 患者以及 23 例 VWF 治疗后患者中进行了比较。

结果

与手动 VWF：RCo 测定法的 7%相比，自动化测定法的实验内和实验间变异系数（分别为 2.3%和 3.8%）得到了改善。在 53 例 VWD 患者中，两种测定法之间存在良好的相关性（r=0.91）。手动 VWF：RCo 的平均值为 0.25IU/ml，自动 VWF：RCo 的平均值为 0.27IU/ml。在 13 例 1 型 VWD 患者中，经治疗（主要使用去氨加压素）后 VWF：RCo 增加，与手动 VWF：RCo 相比，自动 VWF：RCo 增加了 3.9 倍（3.1 倍）。在 13 例 2 型或 3 型 VWD 患者中，经治疗（主要使用浓缩剂）后，自动 VWF：RCo 测定法的增加幅度高于手动测定法（手动 11.9 倍，自动 20.3 倍）。