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猪源凝血因子VIII在获得性血友病患者治疗中的应用:英国的经验。

The use of porcine factor VIII in the treatment of patients with acquired hemophilia: the United Kingdom experience.

作者信息

Morrison A E, Ludlam C A

机构信息

Department of Haematology, Royal Infirmary, Edinburgh, Scotland, United Kingdom.

出版信息

Am J Med. 1991 Nov 4;91(5A):23S-26S. doi: 10.1016/s0002-9343(91)80144-b.

Abstract

Data have been collected by questionnaire on 15 acute bleeding episodes in 12 patients with acquired hemophilia, treated with porcine factor VIII (FVIII). The median initial anti-human FVIII inhibitor level was 40 Bethesda Units (BU)/ml, whereas that against porcine was 1 BU/ml. The mean initial dose of porcine FVIII infused was 54 micrograms/kg, which resulted in a rise of 0.57 IU/mL in the FVIII concentration. Therapy was continued for a mean of 8.5 days, during which time the average number of infusions was 11. Clinical response was rated as good or excellent in 82% of recipients. Side effects were uncommon; only one patient experienced a severe reaction. Following therapy, no increase in antihuman antibody levels was noted; increased levels of antiporcine antibody was detected in only two patients. One patient bled on three further occasions and was successfully retreated with porcine FVIII. Porcine FVIII is a safe and clinically effective treatment for bleeding episodes in acquired hemophilia and should be considered as first-line therapy for patients with low antiporcine FVIII levels.

摘要

通过问卷调查收集了12例获得性血友病患者使用猪源凝血因子VIII(FVIII)治疗15次急性出血事件的数据。初始抗人FVIII抑制剂水平的中位数为40贝塞斯达单位(BU)/毫升,而抗猪源的为1 BU/毫升。输注猪源FVIII的平均初始剂量为54微克/千克,这使得FVIII浓度升高了0.57 IU/mL。治疗平均持续8.5天,在此期间平均输注次数为11次。82%的接受治疗者临床反应评为良好或优秀。副作用不常见;只有1例患者出现严重反应。治疗后,未观察到抗人抗体水平升高;仅在2例患者中检测到抗猪源抗体水平升高。1例患者又发生了3次出血,并成功地再次接受猪源FVIII治疗。猪源FVIII是治疗获得性血友病出血事件的一种安全且临床有效的疗法,对于抗猪源FVIII水平低的患者应考虑作为一线治疗。

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