Increased clearance of von Willebrand factor antigen post-DDAVP in type 1 von Willebrand disease: is it a potential pathogenic process?
Increased clearance of von Willebrand factor antigen post-DDAVP in type 1 von Willebrand disease: is it a potential pathogenic process?
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DOI：
10.1046/j.1538-7836.2003.00359.x
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发表时间：
2003-08-01
影响因子：
10.4
通讯作者：
Bowen, DJ
中科院分区：
文献类型：
--
作者：
Brown, SA;Eldridge, A;Bowen, DJ
血管性血友病因子 (VWF) 清除机制尚不完全清楚。影响VWF清除的因素以及正常的体内清除机制可能与VWF部分缺乏的1型冯维勒布兰德病（VWD）的发病机制有关。为了研究 1 型 VWD 中 VWF 的清除情况，本研究评估了 1 型 VWD 患者和轻度 A 型血友病患者服用 1-脱氨基-8-D-精氨酸加压素 (DDAVP；去氨加压素) 后 VWF 抗原 (t(1/2) VWF:Ag) 的半衰期。迄今为止，已对 20 名个体进行了评估，其中 13 名患有 I 型 VWD，7 名患有轻度 A 型血友病。I 型 VWD 和轻度 A 型血友病组中的中位 t(1/2)、VWF:Ag 分别为 4.6 小时和 9.5 小时。两组t(1/2) VWF:Ag差异有显着性，P < 0.02。数据分析显示，在给予 DDAVP 之前，t(1/2) VWF:Ag 与基线 VWF:Ag 水平之间存在相关性：较低的基线 VWF:Ag 水平与较短的 t(1/2) VWF:Ag 相关。这些数据表明，VWF 清除率增加可能是某些 I 型 VWD 病例的致病机制。
The mechanism of von Willebrand factor (VWF) clearance is not fully understood. The factors that affect VWF clearance, and the normal in vivo mechanism of clearance, may be relevant to the pathogenesis of Type 1 von Willebrand disease (VWD), in which there is a partial deficiency of VWF. In order to investigate the clearance of VWF in Type 1 VWD, the current study assessed the half-life of VWF antigen (t(1/2) VWF:Ag) in Type 1 VWD patients and individuals with mild hemophilia A following the administration of 1-deamino-8-D-arginine vasopressin (DDAVP; desmopressin). To date 20 individuals have been assessed, 13 with Type I VWD and seven with mild hemophilia A. The median t(1/2), VWF:Ag in the Type I VWD and mild hemophilia A groups were 4.6h and 9.5 h, respectively. The difference between the t(1/2) VWF:Ag for the two groups was significant, P < 0.02. Analysis of the data showed a correlation between the t(1/2) VWF:Ag and the baseline VWF:Ag level prior to administration of DDAVP: lower baseline VWF:Ag levels were associated with a shorter t(1/2) VWF:Ag. These data suggest that increased clearance of VWF may be the pathogenic mechanism in some cases of Type I VWD.