Vinazzer H, Stocker K
Blood Coagulation Laboratory, Linz, Austria.
Thromb Res. 1991 Feb 1;61(3):235-41. doi: 10.1016/0049-3848(91)90099-i.
By a series of experiments it could be shown that an activity test of heparin cofactor II (HC II) is only specific after a complete depletion of antithrombin III. Also when dermatan sulfate which does not enhance the action of AT III is used for the activation of HC II there is a considerable influence on the remaining thrombin activity which alters the test results. Furthermore, in a system which contains plasma as well as thrombin the formation of a clot is likely to occur which by its opacity influences photometric results. A chromogenic substrate assay is described which excludes the influence of these variables. This assay was used to examine the activity of HC II in healthy persons as well as in patients. Three members of a family were found who had a heterozygous deficiency of HC II without any history of thrombosis. On the other hand, a total of 16 patients with heterozygous deficiency of HC II suffered from recurrent thromboembolic episodes. For this reason it is assumed that a deficiency of HC II has a certain importance in the occurrence of thrombophilia though it is apparently less thrombogenic than a deficiency of other inhibitors.
通过一系列实验可以表明，只有在抗凝血酶III完全耗尽后，肝素辅因子II（HC II）的活性测试才具有特异性。此外，当使用不增强AT III作用的硫酸皮肤素激活HC II时，对剩余的凝血酶活性有相当大的影响，这会改变测试结果。此外，在一个包含血浆和凝血酶的系统中，很可能会形成凝块，其不透明度会影响光度测定结果。本文描述了一种发色底物测定法，该方法排除了这些变量的影响。该测定法用于检测健康人和患者体内HC II的活性。发现一个家族的三名成员存在HC II杂合性缺乏，且无任何血栓形成病史。另一方面，共有16名HC II杂合性缺乏的患者反复发作血栓栓塞事件。因此，尽管HC II缺乏显然比其他抑制剂缺乏的血栓形成性小，但推测HC II缺乏在血栓形成倾向的发生中具有一定重要性。
