FAMILIAL HEMORRHAGIC TRAIT ASSOCIATED WITH A DEFICIENCY OF A CLOT-PROMOTING FRACTION OF PLASMA
FAMILIAL HEMORRHAGIC TRAIT ASSOCIATED WITH A DEFICIENCY OF A CLOT-PROMOTING FRACTION OF PLASMA
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DOI：
10.1172/jci103109
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发表时间：
1955-01-01
影响因子：
15.9
通讯作者：
COLOPY, JE
中科院分区：
文献类型：
--
作者：
RATNOFF, OD;COLOPY, JE
研究对象（3）的静脉血凝固时间大大延长，但没有明显的出血症状。这种特征显然是家族性的，男女皆有。患者的疾病无法与任何已知的出血性疾病相鉴别。血浆中缺乏正常球蛋白中的一种物质，这种物质会加速正常、缺乏血小板的血浆的凝固。这种促凝成分存在于加热的硫酸钡吸附血清中，既不形成血栓，也不形成血栓形成，并且不会改变凝血酶将纤维蛋白原转化为纤维蛋白的速率，也不会改变组织凝血活酶将凝血酶原转化为凝血酶的速率。据推断，它的作用是在凝血的早期阶段，大概是在流血中血栓形成活性的发展上。该级分似乎不含有任何已知对于血浆血栓形成活性的最佳进化所必需的血浆成分。促血栓成分影响血栓形成活性发展的方法尚未确定。正常天然球蛋白缩短了硅胶涂层管中正常天然血浆的凝血时间，而患者的天然球蛋白则没有效果。血友病天然球蛋白在缩短正常天然血浆的凝血时间方面不如正常球蛋白有效，尽管血友病血浆含有与正常血浆相同量的促凝部分。一种可能的假设是促凝组分发挥其作用与抗血友病因子相关。在观察到第一个患者后，促凝部分被暂时指定为“哈格曼因子”，其缺陷被称为“哈格曼特征”。这3名患者表明，凝血时间可能会延长多年，而不会造成明显伤害。
Patients (3) were studied in whom the clotting time of venous blood was greatly prolonged but without significant hemorrhagic symptoms. The trait was apparently familial and appeared in both sexes. The patients'' disorder could not be identified with any known bleeding disease. The plasma was deficient in a substance found in normal globulin which accelerated the clotting of normal, platelet-deficient plasma. This clot-promoting fraction, present in heated, barium sulfate-adsorbed serum, was neither thrombic or thromboplastic and did not alter the rate of conversion of fibrinogen to fibrin by thrombin, nor of prothrombin to thrombin by tissue thromboplastin. By inferences, its effect was upon an earlier stage of clotting, presumably upon the development of thromboplastic activity in shed blood. The fraction did not appear to contain any of the components of plasma known to be necessary for the optimal evolution of plasma thromboplastic activity. The method by which the clot-promoting fraction influenced the development of thromboplastic activity was not defined. Normal native globulin shortened the clotting time of normal native plasma in silicone-coated tubes, whereas the patients'' native globulin was without effect. Hemophilic native globulin was less effective than the normal in shortening the clotting time of normal native plasma, even though hemophilic plasma contained the same amount of the clot-promoting fraction as normal plasma. One possible hypothesis is that the clot-promoting fraction exerts its action in association with the anti-hemophilic factor. Temporarily the clot-promoting fraction is designated "Hageman factor", and its deficiency "Hageman trait", after the 1st patient observed. The 3 patients indicate that it is possible to have a prolonged clotting time for many years without obvious harm.