Lee H, Nurden A T, Thomaidis A, Caen J P
Br J Haematol. 1981 May;48(1):47-57. doi: 10.1111/j.1365-2141.1981.00047.x.
We have studied the fibrinogen binding to the platelets of four type I thrombasthenic two type II thrombasthenic patients and 15 normal subjects. The amount of [125I]fibrinogen bound to washed platelets in the presence or absence of 10 micrometers ADP was measured and the results expressed as the percentage of the total radioactivity added. The mean value for specific binding to the platelets of normal subjects was 3.7 (range 1--7.25) at 15 min after the addition of ADP. Type I thrombasthenic patients lack glycoproteins (GP) IIb and IIIa in the platelet membrane and intra-platelet fibrinogen. In all four patients studied, little or no specific fibrinogen binding was detected. Platelets of type II thrombasthenic patients contain detectable, although markedly reduced amounts of GP IIb and IIIa, and subnormal or normal levels of fibrinogen. In these platelets, significant specific fibrinogen binding was observed, the values being slightly below or at the lower end of the normal range. Fibrinogen binding to normal platelets was also measured after the addition of an IgG purified from the serum of a multi-transfused thrombasthenic type I patient. This antibody was previously shown to be directed against the GP IIb/IIIa complex as located by crossed immunoelectrophoresis. Specific fibrinogen-binding was blocked and the extent of the inhibition was directly proportional to the concentration of the antibody added.
我们研究了4例I型血小板无力症患者、2例II型血小板无力症患者及15名正常受试者血小板与纤维蛋白原的结合情况。测定了在存在或不存在10微摩尔ADP的情况下，与洗涤过的血小板结合的[125I]纤维蛋白原的量，结果以添加的总放射性的百分比表示。添加ADP后15分钟，正常受试者血小板特异性结合的平均值为3.7（范围1 - 7.25）。I型血小板无力症患者血小板膜和血小板内纤维蛋白原中缺乏糖蛋白（GP）IIb和IIIa。在所研究的所有4例患者中，几乎未检测到特异性纤维蛋白原结合或未检测到。II型血小板无力症患者的血小板含有可检测到的GP IIb和IIIa，尽管其含量明显减少，纤维蛋白原水平低于正常或正常。在这些血小板中，观察到显著的特异性纤维蛋白原结合，其值略低于正常范围或处于正常范围的下限。在添加从一名多次输血的I型血小板无力症患者血清中纯化的IgG后也测量了纤维蛋白原与正常血小板的结合。先前通过交叉免疫电泳显示该抗体针对GP IIb/IIIa复合物。特异性纤维蛋白原结合被阻断，抑制程度与添加的抗体浓度成正比。
