Hay C R, Laurian Y, Verroust F, Preston F E, Kernoff P B
Royal Liverpool Hospital, UK.
Blood. 1990 Sep 1;76(5):882-6.
Home therapy with porcine factor VIIIC was safe and effective when administered to five hemophilic patients over periods of 8 1/2, 6, 4, 3 1/2, and 2 years. No significant transfusion reactions occurred. Before treatment with porcine factor VIIIC, all five had high-level, high-responding anti-human VIIIC inhibitors initially lacking anti-porcine factor VIIIC activity. Although specific anti-porcine VIIIC inhibitors arose in all patients, these were generally transient, and only one patient became refractory to treatment. We believe that porcine factor VIIIC is the treatment of choice in patients whose inhibitors do not cross-react. All five patients lost their original anti-human VIIIC inhibitors after starting treatment with porcine VIIIC, permitting the reintroduction of human VIIIC in three of them. There has been no recurrence of anti-human VIIIC inhibitor activity during 2 to 3 years of regular treatment with human VIIIC in these patients. This suggests that tolerance to human VIIIC has arisen as a result of treatment with porcine VIIIC. Porcine VIIIC may have a role in the desensitization of some factor VIIIC inhibitor patients.
对5名血友病患者进行为期8.5年、6年、4年、3.5年和2年的猪源凝血因子VIIIC家庭治疗是安全有效的。未发生明显的输血反应。在用猪源凝血因子VIIIC治疗前，所有5名患者最初均有高水平、高反应性的抗人VIIIC抑制剂，且最初缺乏抗猪源凝血因子VIIIC活性。尽管所有患者均出现了特异性抗猪VIIIC抑制剂，但这些抑制剂通常是短暂的，只有1名患者对治疗产生了耐药性。我们认为，猪源凝血因子VIIIC是抑制剂无交叉反应患者的首选治疗方法。所有5名患者在开始使用猪源VIIIC治疗后均失去了原有的抗人VIIIC抑制剂，其中3名患者得以重新使用人VIIIC。在这些患者接受人VIIIC常规治疗的2至3年中，未出现抗人VIIIC抑制剂活性的复发。这表明，由于使用了猪源VIIIC治疗，对人VIIIC产生了耐受性。猪源VIIIC可能在一些凝血因子VIIIC抑制剂患者的脱敏治疗中发挥作用。
