Diéval J, Nguyen G, Gross S, Delobel J, Kruithof E K
Laboratory of Hematology, University Hospital, Amiens, France.
Blood. 1991 Feb 1;77(3):528-32.
A 36-year-old patient was investigated for a lifelong history of epistaxis and delayed bleeding after minor surgeries. Deficiencies or abnormalities of the coagulation system, of platelet function, or of factor XIII and alpha-2-antiplasmin were excluded. Consistently, however, over a period of 7 years, a high basal euglobulin fibrinolytic activity was observed that was characterized by a high tissue-type plasminogen activator (t-PA) activity, normal t-PA antigen, and undetectable plasminogen activator inhibitor type-1 (PAI-1) antigen and activity. The high specific activity of t-PA (640,000 IU/mg) and the minimal amounts of t-PA/PAI-1 complexes detected by fibrin zymography suggest that in this patient all t-PA was active. This is in striking contrast to normal plasma, where the majority of t-PA is complexed to PAI-1. Thus, in this patient, a severe deficiency of PAI-1 is associated with a delayed type bleeding tendency. Our observation underscores the importance of plasma PAI-1 for the stabilization of the hemostatic plug.
一名36岁患者因终生鼻出血及小手术后出血延迟而接受检查。排除了凝血系统、血小板功能、因子XIII和α-2-抗纤溶酶的缺乏或异常。然而，在7年的时间里，持续观察到高基础优球蛋白纤维蛋白溶解活性，其特征为高组织型纤溶酶原激活物（t-PA）活性、正常t-PA抗原，且未检测到纤溶酶原激活物抑制剂-1（PAI-1）抗原及活性。t-PA的高比活性（640,000 IU/mg）以及纤维蛋白酶谱检测到的极少量t-PA/PAI-1复合物表明，该患者体内所有t-PA均具有活性。这与正常血浆形成鲜明对比，在正常血浆中，大多数t-PA与PAI-1结合。因此，在该患者中，PAI-1严重缺乏与延迟性出血倾向相关。我们的观察结果强调了血浆PAI-1对止血栓稳定的重要性。
