Aznar J, Estellés A, Vila V, Regañón E, España F, Villa P
Thromb Haemost. 1984 Oct 31;52(2):196-200.
A family with "in vitro" increased red-cell fall out from the blood clot was studied. One member of the family (JVM) had a clinical history of hemorrhages after minor trauma or dental extractions. Routine coagulation and platelet function were normal except for the fibrinogen level which was slightly low in several members. The antigenic as well as functional evaluation of factor XIII was within normal limits. No factor XIII inhibitors were present. An increase in the clot permeability index was observed in most family members. The study of the fibrinolytic system showed an enhanced lysis of euglobulins, a normal plasminogen value, normal level of fibrin/ogen degradation products, normal fibrinolytic inhibitors, and an increase in the activity of the plasminogen activator. The activity of this activator was inhibited by an antiserum against tissue-type plasminogen activator. The t-pA inhibitor was in the normal range. It is concluded that the family studied in this paper shows familial alteration in the fibrinolytic system due to an excess of plasminogen activator immunologically related to that in human tissue.
对一个“体外”血液凝块红细胞脱落增加的家族进行了研究。该家族的一名成员(JVM)有轻微创伤或拔牙后出血的临床病史。除了几名成员的纤维蛋白原水平略低外,常规凝血和血小板功能正常。因子 XIII 的抗原及功能评估均在正常范围内。不存在因子 XIII 抑制剂。在大多数家族成员中观察到凝块通透性指数增加。纤维蛋白溶解系统研究显示优球蛋白溶解增强、纤溶酶原值正常、纤维蛋白/原降解产物水平正常、纤维蛋白溶解抑制剂正常,且纤溶酶原激活剂活性增加。该激活剂的活性被抗组织型纤溶酶原激活剂的抗血清抑制。组织型纤溶酶原激活剂抑制剂在正常范围内。结论是,本文研究的家族显示出纤维蛋白溶解系统的家族性改变,这是由于与人体组织中纤溶酶原激活剂免疫相关的过量激活剂所致。