全血瑞斯托霉素诱导的血小板阻抗聚集测定不能反映 1 型血管性血友病患者的临床严重程度。
Whole blood ristocetin-induced platelet impedance aggregometry does not reflect clinical severity in patients with type 1 von Willebrand disease.
机构信息
Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan.
The Course of Hemophilia Education, Nara Medical University, Kashihara, Nara, Japan.
出版信息
Haemophilia. 2019 May;25(3):e174-e179. doi: 10.1111/hae.13725. Epub 2019 Mar 13.
BACKGROUND
The haemorrhagic phenotype in patients with von Willebrand disease (VWD) is heterogeneous, and assays of von Willebrand factor ristocetin cofactor activity (VWF:RCo) do not always reflect clinical severity, especially in those individuals classed as type 1 VWD. Recent studies have shown that whole blood ristocetin-induced platelet agglutination (WB-RIPA) using an easy-to-use analyzer, Multiplate® platelet impedance technique, could be informative as a diagnostic test in VWD, although inconsistencies were evident in patients with the type 1 disorder, possibly associated with clinical symptoms.
AIM
To investigate the relationship between WB-RIPA, bleeding scores (BS) and VWF-related measurements in type 1 VWD.
METHODS
WB-RIPA assay using the Multiplate® was performed using whole blood from 55 patients with type 1 VWD. BS was determined using a standardized questionnaire.
RESULTS
WB-RIPA values were significantly lower in type 1 VWD than in healthy controls (P < 0.0001). Weak correlations were apparent between WB-RIPA and VWF:RCo or VWF antigen (VWF:Ag; r = 0.22 or 0.28, respectively). There were significant differences in VWF:RCo (P = 0.036) and VWF:Ag (P = 0.0013) between patients with BS ≥4 (defined as abnormal bleeding tendency) and BS <4 (defined as no abnormal bleeding tendency), respectively. However, no significant difference was observed in WB-RIPA between the BS ≥4 group and BS <4 group. Overall, VWD patients with a WB-RIPA level >70 U did not seem to have an abnormal bleeding tendency, but low levels of WB-RIPA did not correlate with BS.
CONCLUSION
WB-RIPA did not reflect clinical severity in type 1 VWD patients.
背景
血管性血友病（VWD）患者的出血表型存在异质性，并且 von Willebrand 因子瑞斯托菌素辅因子活性（VWF：RCo）的检测并不总是反映临床严重程度，尤其是在那些被归类为 1 型 VWD 的患者中。最近的研究表明，使用易于使用的分析仪全血瑞斯托菌素诱导的血小板聚集（WB-RIPA）Multiplate®血小板阻抗技术可以作为 VWD 的诊断测试提供信息，尽管在 1 型疾病患者中存在明显的不一致性，可能与临床症状有关。
目的
研究 1 型 VWD 中 WB-RIPA、出血评分（BS）和 VWF 相关测量值之间的关系。
方法
使用 55 例 1 型 VWD 患者的全血进行 WB-RIPA 检测。使用标准化问卷确定 BS。
结果
与健康对照组相比，1 型 VWD 患者的 WB-RIPA 值显著降低（P<0.0001）。WB-RIPA 与 VWF：RCo 或 VWF 抗原（VWF：Ag）之间存在明显的弱相关性（r 分别为 0.22 或 0.28）。VWF：RCo（P=0.036）和 VWF：Ag（P=0.0013）在 BS≥4（定义为异常出血倾向）和 BS<4（定义为无异常出血倾向）的患者之间存在显著差异。然而，BS≥4 组和 BS<4 组之间的 WB-RIPA 无显著差异。总体而言，WB-RIPA 水平>70 U 的 VWD 患者似乎没有异常出血倾向，但低水平的 WB-RIPA 与 BS 无关。
结论
WB-RIPA 不能反映 1 型 VWD 患者的临床严重程度。
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