Eller T, Brauer P, Albert J, Mayer J, Keller F
Central Laboratory of the Medical University Hospital, Würzburg, FRG.
Thromb Res. 1992 Mar 1;65(4-5):631-40. doi: 10.1016/0049-3848(92)90212-s.
The von Willebrand factor (vWF) is found in plasma and in platelets. The concentration and multimeric composition of the vWF in platelets of 160 patients with bleeding tendency were examined since very little is known about the platelet vWF. For quantitative analysis of the platelet vWF, a modified ELISA was established. A reference range from 70%-130% of platelet vWF concentration considered normal was established by examining 80 healthy blood donors. 16.9% of the 160 patients showed a decreased vWF concentration in platelets only, while all the other coagulation parameters were normal. 3 of our patients belong to the same family and suggesting an autosomal dominant genetic transmission for the von Willebrand disease type 1-3. The data also suggests, that a quantitative and qualitative analysis of the vWF in plasma and platelets is required for an exact diagnosis of the von Willebrand disease.
血管性血友病因子（vWF）存在于血浆和血小板中。由于对血小板vWF了解甚少，因此对160例有出血倾向患者血小板中的vWF浓度和多聚体组成进行了检测。为了对血小板vWF进行定量分析，建立了一种改良的酶联免疫吸附测定法（ELISA）。通过检测80名健康献血者，确定了血小板vWF浓度正常范围为70%-130%。160例患者中有16.9%仅表现为血小板vWF浓度降低，而所有其他凝血参数均正常。我们的3例患者来自同一家族，提示为1-3型血管性血友病的常染色体显性遗传。这些数据还表明，要准确诊断血管性血友病，需要对血浆和血小板中的vWF进行定量和定性分析。
