Studt J D, Budde U, Schneppenheim R, Eisert R, von Depka Prondzinski M, Ganser A, Barthels M
Department of Haematology and Oncology, Hannover Medical School, Carl-Neuberg-Str. 1, D-30625 Hannover, Germany.
Am J Clin Pathol. 2001 Oct;116(4):567-74. doi: 10.1309/75CQ-V7UX-4QX8-WXE7.
The analysis of von Willebrand factor (vWF) multimers is an important laboratory tool for distinguishing among the numerous subtypes of von Willebrand disease (vWD). Comparability and reproducibility of this method are insufficient; standardization and external references are pending. Interlaboratory comparison of results therefore may be difficult. We applied densitometry to obtain a reproducible quantification of vWF multimer patterns in healthy donors, patients with vWD variants, and factor VIII/vWF concentrates to improve the reproducibility and comparability of vWF multimer analysis. Multimers were separated and visualized luminographically on x-ray films. Films were scanned and evaluated by densitometry. The variation inherent in vWF multimer analysis and the range of the normal could be quantified. In vWD variants and factor VIII/vWF concentrates, densitometry could quantify and visualize alterations of vWF multimer patterns and facilitate their comparison. Densitometry permits a precise quantitative comparison of sample patterns to a reference plasma. It could be a valuable tool offering reproducible quantification and additional visualization of normal and pathologic vWF multimer patterns, facilitating their comparison and contributing to a standardization of vWF multimer analysis.
血管性血友病因子（vWF）多聚体分析是区分多种血管性血友病（vWD）亚型的重要实验室工具。该方法的可比性和可重复性不足；标准化和外部参考仍悬而未决。因此，实验室间结果的比较可能很困难。我们应用光密度测定法对健康供体、vWD变异型患者以及凝血因子VIII/vWF浓缩物中的vWF多聚体模式进行可重复的定量分析，以提高vWF多聚体分析的可重复性和可比性。多聚体经分离后在X光片上进行发光成像显示。扫描X光片并通过光密度测定法进行评估。vWF多聚体分析中固有的变异以及正常范围可以被量化。在vWD变异型和凝血因子VIII/vWF浓缩物中，光密度测定法可以量化并显示vWF多聚体模式的改变，便于进行比较。光密度测定法能够将样本模式与参考血浆进行精确的定量比较。它可能是一种有价值的工具，可为正常和病理性vWF多聚体模式提供可重复的定量分析和额外的可视化显示，便于比较，并有助于vWF多聚体分析的标准化。
