Gadner H, Odenwald E, Jarofke R, Riehm H
Klin Wochenschr. 1977 Dec 1;55(23):1165-71. doi: 10.1007/BF01478054.
This report describes two patients with haemophilia A who developed a transient thrombocytopathy with haemorrhagic diathesis during post-operative high-dose replacement therapy with antihaemophilic globulin. At the time of the bleeding the factor VIII-activity was in the normal range in both patients. The fibrinogen level, however, was elevated to 1700 mg-% and the factor VIII-associated antigen rose to more than 6-fold. At no time of replacement therapy with antihaemophilic globulin could either fibrinogen split products or fragments of the factor VIII-protein be detected by the usual methods. In view of the results of the thrombocyte aggregation experiments the authors postulate a disturbance of platelet function at the level of the membrane surface due to an overload of increased amounts of circulating proteins. Both the possible interference of dialysable factor VIII-components and the role of immunpathologic phenomena are discussed.
本报告描述了两名甲型血友病患者，他们在术后接受高剂量抗血友病球蛋白替代治疗期间出现了伴有出血素质的短暂血小板病。出血时，两名患者的因子VIII活性均在正常范围内。然而，纤维蛋白原水平升高至1700mg-%，且因子VIII相关抗原升至6倍以上。在用抗血友病球蛋白进行替代治疗的任何时候，用常规方法均未检测到纤维蛋白原裂解产物或因子VIII蛋白片段。鉴于血小板聚集实验的结果，作者推测由于循环蛋白量增加导致膜表面水平的血小板功能紊乱。文中讨论了可透析因子VIII成分的可能干扰以及免疫病理现象的作用。
