The pathogenesis of hereditary hæmophilia
The pathogenesis of hereditary hæmophilia
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遗传性血友病的发病机制
DOI：
10.1002/path.1700150402
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发表时间：
2010
期刊：
影响因子：
--
通讯作者：
T. Addis
中科院分区：
文献类型：
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作者：
T. Addis
关于遗传性血友病病因的初步研究的详细情况发表在1910年《医学季刊》第四卷上。十三号。得出以下结论：（1）血液凝固延迟。(2)临床症状的严重程度与凝血功能障碍的程度成正比。(3)血栓激酶是一种来源于组织和血液细胞成分的物质，它启动凝血过程。伤口中血液凝固的速度取决于加入的凝血酶的量，血友病患者的血液比正常人的血液需要大量的凝血酶才能迅速凝固。因此，它是关于在hremophilic的伤口中的血液凝固只有在血栓激酶的浓度是最高的，即在伤口的两侧。但是血栓阻止了更多的凝血酶激酶到达伤口中心的血液，如果从外周血凝块中释放的凝血酶量不足以凝固中心血液，出血可能会无限期地持续下去。因此，从血液凝固性缺陷的角度来看，嗜热性贫血患者长期持续的出血是可以解释的。(4)嗜热细胞并不比正常人更容易出血。它们之间的区别不在于出血的发生，而在于出血的量。因此，没有必要假定嗜热性毛细血管的特殊脆弱性，以解释他们所遭受的容易产生的瘀伤和所谓的”自发性”出血。由于这些原因，并由于没有证据表明存在任何其他病理因素能够解释这种情况，血液凝固性差被认为是遗传性血友病的近因。在预备曲中
THE details of a preliminary inquiry into the cause of hereditary hemophilia were published in the Quarterly Journal of Medicim, 1910, vol. iv. No. 13. The following conclusions were arrived at:(1) The coagulation of the blood is delayed.(2) The severity of the clinical symptoms is directly proportional to the degree of the defect ill coagulation.(3) Thrombokinase, a substance derived from the cellular elements of the tissues and of the blood, initiates the procesa of coagulation. The rapidity of the coagulation of blood in; I wound depends on the amount of thrombokinase added to it. A much larger amount of thrombokinase is required to produce the rapid coagulation of hemophilic than of normal blood. It thus comes about that the blood in a wound in a hremophilic coagulates only where the concentration of thrombokinase is highest, ie on the sides of the wound. But thia clot prevents any more thrombokinase from reaching the blood in the centre of the wound, and if the amount of thrombin liberated from this peripheral clot is insufficient to coagulate the central blood, hamorrhage may con tinue indefinitely. The long-continued hzmorrhages seen in hzmophilia are thus explicable from the point of view of the defective coagulability of the blood alone.(4) Haemorrhage is no more easily produced in a htzmophilic than in a normal person. The distinction between them lies not in the occurrence, but in the amount, of the bleeding. There is thus no need to assume a special friability of the capillaries in hremophilia in order to explain the easily produced bruising and the so-called" spontaneous" haemorrhages from which they suffer. For these reasons, and because there ia no evidence of the presence of any other pathological factor capable of explaining the condition, the de6ciency in the coagulability of the blood was accepted as the proximate cause of hereditary hemophilia. In the preseut