Takahashi H, Shibata A
Thromb Haemost. 1985 Apr 22;53(2):204-7.
It has been shown that platelets from patients with platelet-type von Willebrand's disease (vWD) agglutinate upon the addition of human von Willebrand factor (vWF) in the absence of ristocetin or botrocetin, suggesting that platelet membrane receptors for human vWF is abnormal. The present work reports the platelet agglutinability on stimulation with bovine vWF in platelet-type vWD. Platelets in patient platelet-rich plasma or washed platelet suspensions and patient platelets treated with formalin agglutinated in the presence of markedly lower concentrations of bovine vWF than those required for normal platelets. This finding provides additional evidence that platelet-type vWD platelets have abnormal expression of binding sites for vWF on their surface, and supports that platelet receptors for bovine vWF are identical or very close to those for human vWF.
研究表明,血小板型血管性血友病(vWD)患者的血小板在添加人血管性血友病因子(vWF)且不存在瑞斯托菌素或蛇毒凝血酶的情况下会发生凝集,这表明人vWF的血小板膜受体异常。本研究报告了血小板型vWD患者的血小板在牛vWF刺激下的凝集能力。与正常血小板相比,富含患者血小板的血浆或洗涤后的血小板悬液中的血小板以及经福尔马林处理的患者血小板,在明显更低浓度的牛vWF存在下就会发生凝集。这一发现进一步证明血小板型vWD患者的血小板表面vWF结合位点表达异常,并支持牛vWF的血小板受体与人vWF的血小板受体相同或非常接近。