Brody J I
Am J Med Sci. 1975 Jan-Feb;269(1):19-24. doi: 10.1097/00000441-197501000-00003.
This report describes three patients with a prolonged bleeding time and reduced platelet retention on glass beads, two of the three established criteria for conventional von Willebrand's disease (vWd). However, the third standard, the diminution of factor VIII, was replaced by a decrease of factor IX or XI in the first two instances, respectively, and combined factor VII and IX deficiency was present in the last patient. These associations suggest that the vW (bleeding time/platelet retention) factor is not necessarily homogeneous, may not be dependent on a single genetic determinant, and may be present in a molecular complex composed not only of factor VIII but of other blood clotting proteins as well. Although simulating classic vW, these homologues may more appropriately be called von Willebrand syndromes. Finally, unusual as they may be, their clinical recognition is important to insure that such patients receive not only factor VIII concentrates in treatment since these materials do not contain factors IX and XI and may not even be a suitable source of platelet retention factor under these modified circumstances.
本报告描述了三名出血时间延长且玻璃珠上血小板滞留率降低的患者，这是传统血管性血友病（vWd）的三项既定标准中的两项。然而，三项标准中的第三项，即因子VIII减少，在前两例中分别被因子IX或XI减少所取代，最后一名患者存在联合因子VII和IX缺乏。这些关联表明，血管性血友病（出血时间/血小板滞留）因子不一定是同质的，可能不依赖于单一的遗传决定因素，并且可能存在于不仅由因子VIII而且还由其他血液凝固蛋白组成的分子复合物中。尽管模拟了经典的血管性血友病，但这些同源物可能更恰当地称为血管性血友病综合征。最后，尽管它们可能不常见，但对其进行临床识别很重要，以确保此类患者在治疗中不仅接受因子VIII浓缩物，因为这些材料不含因子IX和XI，在这些改变的情况下甚至可能不是血小板滞留因子的合适来源。
