Tosetto Alberto, Castaman Giancarlo, Rodeghiero Francesco
Hematology Department, S. Bortolo Hospital, 36100 Vicenza, Italy.
Blood Rev. 2007 Mar;21(2):89-97. doi: 10.1016/j.blre.2006.04.002. Epub 2006 Jun 13.
Quantification of the bleeding severity in von Willebrand disease (VWD) by use of a bleeding score (BS) could be a useful tool toward a more objective diagnosis. The BS is computed as the sum of arbitrary grades of bleeding severity for several hemorrhagic symptoms, and in normal subjects is nearly always lower than 3. In the diagnosis of type 1 VWD, the use of the BS provided a high specificity (99.1%) and a satisfactory sensitivity (64.2%) in a multicenter, retrospective study. The BS is strongly related to all available measurements of von Willebrand factor activity, including the PFA-100 closure time. The BS could be used to assess the influence on bleeding symptoms of other biological factors in VWD. Therefore, BS represent a promising clinical tool for the analysis of bleeding in VWD, although further validation is warranted before an extensive use in clinical practice could be purported.
使用出血评分（BS）对血管性血友病（VWD）的出血严重程度进行量化，可能是实现更客观诊断的有用工具。出血评分是通过对几种出血症状的出血严重程度的任意分级求和计算得出的，在正常受试者中几乎总是低于3。在1型血管性血友病的诊断中，在一项多中心回顾性研究中，出血评分具有较高的特异性（99.1%）和令人满意的敏感性（64.2%）。出血评分与血管性血友病因子活性的所有可用测量指标密切相关，包括PFA-100封闭时间。出血评分可用于评估血管性血友病中其他生物学因素对出血症状的影响。因此，出血评分是分析血管性血友病出血情况的一种有前景的临床工具，尽管在广泛应用于临床实践之前还需要进一步验证。
