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[血液透析动静脉内瘘术后获得性2A型血管性血友病?]

[Acquired von Willebrand's disease type 2A following arteriovenous fistula for haemodialysis?].

作者信息

Leimkühler K, Bach F, Kuhlmann S, Feidicker B, Heim M U, Susemihl C, Schmidt B, Mertzlufft F

机构信息

Klinik für Anästhesiologie, Intensiv-, Notfall-, Transfusionsmedizin und Schmerztherapie, Evangelisches Krankenhaus Bielefeld.

出版信息

Hamostaseologie. 2011 May 2;31(2):118-22. doi: 10.5482/ha-1129. Epub 2010 Dec 9.

Abstract

Acquired von Willebrand's disease (aVWD) is considered to be an underestimated cause of unexplained bleeding. Adsorption of von Willebrand factor (VWF) to tumour cells or hydroxyethyl starch and elimination of VWF by autoantibodies as well as shear stress-induced mechanical alteration of VWF with concomitant cleavage by enzymes may lead to an acquired deficiency of VWF and a bleeding disorder. We report a 39-year-old woman who developed spontaneous bleeding five years after surgical creation of an arteriovenous fistula (AVF) for haemodialysis treatment. AVWD type 2A was diagnosed after successful renal transplantation. One year after surgical closure of the AVF, the aVWD could not be verified again. Thus, the aVWD may have developed because of altered blood flow and shear stress inside the arteriovenous fistula.

摘要

获得性血管性血友病(aVWD)被认为是不明原因出血的一个被低估的原因。血管性血友病因子(VWF)吸附到肿瘤细胞或羟乙基淀粉上,以及自身抗体对VWF的清除,还有剪切应力诱导的VWF机械性改变并伴随酶切,都可能导致获得性VWF缺乏和出血性疾病。我们报告一名39岁女性,在为血液透析治疗手术创建动静脉内瘘(AVF)五年后出现自发性出血。成功肾移植后诊断为2A型获得性血管性血友病。动静脉内瘘手术闭合一年后,无法再次证实存在aVWD。因此,aVWD可能是由于动静脉内瘘内血流和剪切应力改变而发生的。

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