Knöbl P, Lechner K
Department of Medicine I, University of Vienna, Austria.
Baillieres Clin Haematol. 1998 Jun;11(2):305-18. doi: 10.1016/s0950-3536(98)80050-4.
One hundred and five cases of factor V inhibitors were published between 1955 and 1997. According to pathogenesis, factor V inhibitor patients can be divided into five groups: patients exposed to bovine thrombin; patients after surgery without exposure to bovine proteins; miscellaneous associated conditions; 'idiopathic' inhibitors; inhibitors in congenital factor V deficiency. The clinical and biochemical properties are described. The overall prognosis of factor V inhibitors is good, but there are differences among the five groups with the best prognosis in patients exposed to bovine thrombin and the worst prognosis in 'idiopathic' inhibitors. Only a few treatment options are available. Immunoadsorption and plasmapheresis seem to be the most effective methods for therapy of acute bleeding. Many inhibitors disappear spontaneously and it is uncertain whether an immunosuppressive treatment hastens the disappearance of the inhibitor.
1955年至1997年间共发表了105例凝血因子V抑制剂病例。根据发病机制,凝血因子V抑制剂患者可分为五组:接触牛凝血酶的患者;术后未接触牛蛋白的患者;其他相关情况;“特发性”抑制剂;先天性凝血因子V缺乏症患者的抑制剂。文中描述了其临床和生化特性。凝血因子V抑制剂的总体预后良好,但五组之间存在差异,接触牛凝血酶的患者预后最佳,“特发性”抑制剂患者预后最差。可用的治疗方法很少。免疫吸附和血浆置换似乎是治疗急性出血最有效的方法。许多抑制剂会自发消失,免疫抑制治疗是否能加速抑制剂的消失尚不确定。