Department of Medicine, University of Padua Medical School, Via Ospedale 105, 35128, Padua, Italy.
Division of Medicine, Padua City Hospital, Padua, Italy.
J Thromb Thrombolysis. 2018 Jan;45(1):135-141. doi: 10.1007/s11239-017-1559-0.
The concept of resistance in blood coagulation has become important. In the past it was limited to the resistance shown by some patients to heparin, coumarin or aspirin. Subsequently, it was demonstrated that a mutation in a single clotting factor, FV, showed resistance to activated protein C. Since activated protein C is supposed to downregulate aFV and aFVIII, their persistence in the circulation gives origin to a hypercoagulable state. Recently antithrombin resistance has been defined. Several prothrombin abnormalities (dysprothrombinemias) have been shown to be resistant to the action of antithrombin. This is associated with the occurrence of a trombophilic state. Prothrombin may therefore be associated like FV with both a bleeding condition (prothrombin deficiency) and a thrombophilic state (some dysprothrombinemias). Finally, thrombomodulin resistance has been defined in liver cirrhosis. These patients often show an increased ratio between FVIII levels and protein C. This imbalance may be partly responsible for the frequent presence of portal vein thrombosis seen in these patients. All these studies have greatly increased the complexity of the clotting mechanisms and interactions. They have cast light on clinical events which had remained unknown or ill-defined.
抗凝血的概念已变得很重要。过去,它仅局限于某些对肝素、香豆素或阿司匹林有抗药性的患者。随后,研究表明凝血因子 FV 的单一突变可导致对活化蛋白 C 的抗药性。由于活化蛋白 C 理应下调 aFV 和 aFVIII,它们在循环中的持续存在导致高凝状态。最近已确定了抗凝血酶的抗药性。已发现几种凝血酶原异常(异常凝血酶原血症)对抗凝血酶的作用有抗药性。这与血栓形成倾向有关。因此,凝血酶原可能像 FV 一样,与出血状态(凝血酶原缺乏症)和血栓形成倾向(某些异常凝血酶原血症)都有关联。最后,在肝硬化中已确定了血栓调节蛋白的抗药性。这些患者常显示 FVIII 水平与蛋白 C 的比值增加。这种失衡可能部分解释了这些患者中常见的门静脉血栓形成。所有这些研究大大增加了凝血机制和相互作用的复杂性。它们阐明了一些以前未知或定义不明确的临床事件。