Feinstein D I, Rapaport S I, McGehee W G, Patch M J
J Clin Invest. 1970 Aug;49(8):1578-88. doi: 10.1172/JCI106375.
A patient who had received multiple transfusions for complications of acute hemorrhagic pancreatitis developed a potent factor V anticoagulant with bleeding due to defective hemostasis. Despite its potency, the anticoagulant disappeared within 15 days of its first manifestation. A second patient with adenocarcinoma of the colon developed an anticoagulant to factor V postoperatively after a single blood transfusion. The anticoagulants appeared to react stoichiometrically with factor V in normal plasma in vitro. They had the physicochemical properties of immunoglobulins, and their activity was neutralized by antihuman immunoglobulin antiserum. One anticoagulant appeared to be slightly more active against homologous than against autologous factor V, but it also inhibited heterologous factor V. Both anticoagulants progressively inactivated intrinsic prothrombin activator formed from normal reagents in the incubation mixture of the thromboplastin generation test, thus confirming that factor V is required for the effective action of the intrinsic prothrombin activator. Since the anticoagulants were immunoglobulins whose activity was consumed in their reaction with factor V, consumption of anticoagulant activity was used to detect factor V antigenic material in test materials. Human serum without factor V clotting activity was found to consume anticoagulant activity, i.e., to contain inactive factor V antigenic material. Plasma from two patients with hereditary factor V deficiency (parahemophilia) failed to consume significant anticoagulant activity. Thus, the lack of factor V activity in these patients represents a deficiency of factor V molecules rather than the synthesis of a defective molecule with impaired clotting activity.
一名因急性出血性胰腺炎并发症接受多次输血的患者，因止血功能缺陷而产生了一种强效的因子V抗凝剂并伴有出血。尽管其效力很强，但该抗凝剂在首次出现后的15天内就消失了。第二名患有结肠癌的患者在单次输血后术后产生了针对因子V的抗凝剂。这些抗凝剂在体外似乎能与正常血浆中的因子V发生化学计量反应。它们具有免疫球蛋白的物理化学性质，其活性可被抗人免疫球蛋白抗血清中和。一种抗凝剂对同源因子V的活性似乎比对自身因子V的活性略高，但它也能抑制异源因子V。两种抗凝剂都能在凝血活酶生成试验的孵育混合物中逐渐灭活由正常试剂形成的内源性凝血酶原激活物，从而证实内源性凝血酶原激活物的有效作用需要因子V。由于这些抗凝剂是免疫球蛋白，其活性在与因子V的反应中被消耗，因此利用抗凝剂活性的消耗来检测测试材料中的因子V抗原物质。发现不含因子V凝血活性的人血清会消耗抗凝剂活性，即含有无活性的因子V抗原物质。两名遗传性因子V缺乏症（副血友病）患者的血浆未能消耗显著的抗凝剂活性。因此，这些患者中因子V活性的缺乏代表了因子V分子的缺乏，而不是合成了具有受损凝血活性的缺陷分子。
