Department of Medicine, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106.
J Clin Invest. 1975 May;55(5):1082-9. doi: 10.1172/JCI108009.
The prolonged partial thromboplastin time observed in the plasma of a 71-yr-old asymptomatic man was related to the deficiency of a hitherto unrecognized agent. The patient's plasma also exhibited impaired surface-mediated fibrinolysis and esterolytic activity and impaired generation of kinins and of the property enhancing vascular permeability designated PF/Dil. The patient's plasma contained normal amounts of all known clotting factors except Fletcher factor (a plasma prekallikrein) which was present at a concentration of 10-15% of pooled normal plasma. Fletcher trait plasma, however, contained normal amounts of the agent missing from the patient's plasma and corrected the defects in clotting, fibrinolysis, and vascular permeability. Fletcher trait plasma was less effective in correcting generation of kinins and esterolytic activity, presumably because of the patient's partial deficiency of prekallikrein. The site of action of the factor deficient in the patient's plasma appeared to be subsequent to the activation of Hageman factor and plasma prekallikrein. A fraction of normal plasma, devoid of other clotting factors, corrected the defect in clotting in the patient's plasma; a similar fraction of the patient's plasma did not correct this abnormality. No evidence yet exists pointing to the familial nature of the patient's defect. Tentatively, the patient's disorder may be referred to by his surname as Fitzgerald trait, and the agent apparently deficient in his plasma as Fitzgerald factor.
一位 71 岁无症状男性的血浆中出现了延长的部分凝血活酶时间,这与一种以前未被识别的因子缺乏有关。该患者的血浆还表现出表面介导的纤维蛋白溶解和酯酶活性受损,以及激肽生成和血管通透性增强因子(PF/Dil)生成受损。该患者的血浆中除 Fletcher 因子(血浆前激肽释放酶)外,含有所有已知凝血因子的正常量,Fletcher 因子的浓度为正常混合血浆的 10-15%。然而,Fletcher 特征性血浆中含有患者血浆中缺失的因子的正常量,并纠正了凝血、纤维蛋白溶解和血管通透性的缺陷。Fletcher 特征性血浆在纠正激肽生成和酯酶活性方面的效果较差,可能是因为患者存在部分前激肽释放酶缺乏。患者血浆中缺乏的因子的作用部位似乎在 Hageman 因子和血浆前激肽释放酶激活之后。正常血浆的一部分,没有其他凝血因子,纠正了患者血浆中的凝血缺陷;患者血浆的类似部分不能纠正这种异常。目前尚无证据表明患者的缺陷具有家族性质。暂时可以将患者的疾病称为 Fitzgerald 特征,而其血浆中明显缺乏的因子称为 Fitzgerald 因子。