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猪源凝血因子VIII在治疗伴有抑制物的血友病患者中的疗效。

Efficacy of porcine factor VIII in the management of haemophiliacs with inhibitors.

作者信息

Ciavarella N, Antoncecchi S, Ranieri P

出版信息

Br J Haematol. 1984 Dec;58(4):641-8. doi: 10.1111/j.1365-2141.1984.tb06111.x.

DOI:10.1111/j.1365-2141.1984.tb06111.x
PMID:6440588
Abstract

We report our experience with polyelectrolyte-fractionated highly purified porcine factor VIII concentrate (Hyate:C). Porcine factor VIII concentrate was used to treat 14 haemorrhagic episodes including seven severe haemarthroses, three severe haematomata, two episodes of oral bleeding, one traumatic and the other after multiple dental extractions and two life-threatening intestinal haemorrhages. Altogether 60 infusions of Hyate:C were given to five haemophiliacs with inhibitors. At the time of the first infusion with porcine factor VIII the anti-human inhibitor level ranged from 60 to 2.5 modified Bethesda units/ml (MBU 4 h incubation). The porcine cross-reactivity of these inhibitors was 25.6% and 5.4% of the human inhibitor level in two patients and scarcely measurable in the other three patients. We injected an amount of porcine factor VIII concentrate corresponding to the calculated porcine neutralizing units plus the units required for haemostasis. The clinical efficacy was excellent and no adverse effects were encountered, apart from two episodes of mild pyrogenic reactions well controlled by hydrocortisone. The anamnestic antibody response against human factor VIII was of slight degree in all cases, while the porcine cross-reactivity showed no significant variation. Our results emphasize the role of porcine factor VIII in the treatment of haemophiliacs with inhibitors.

摘要

我们报告了使用聚电解质分级分离的高度纯化猪源凝血因子VIII浓缩物(Hyate:C)的经验。猪源凝血因子VIII浓缩物用于治疗14次出血事件,包括7次严重关节积血、3次严重血肿、2次口腔出血、1次外伤后出血和另1次多次拔牙后出血,以及2次危及生命的肠道出血。总共对5名有抑制剂的血友病患者输注了60次Hyate:C。首次输注猪源凝血因子VIII时,抗人抑制剂水平在60至2.5改良贝塞斯达单位/毫升(4小时孵育的MBU)之间。这些抑制剂对猪源的交叉反应性在两名患者中分别为人类抑制剂水平的25.6%和5.4%,在其他三名患者中几乎无法测量。我们注射了相当于计算出的猪源中和单位加上止血所需单位量的猪源凝血因子VIII浓缩物。临床疗效极佳,除了两例轻度热原反应经氢化可的松良好控制外,未出现不良反应。在所有病例中,针对人凝血因子VIII的回忆性抗体反应程度轻微,而猪源交叉反应性无显著变化。我们的结果强调了猪源凝血因子VIII在治疗有抑制剂的血友病患者中的作用。

相似文献

1
Efficacy of porcine factor VIII in the management of haemophiliacs with inhibitors.猪源凝血因子VIII在治疗伴有抑制物的血友病患者中的疗效。
Br J Haematol. 1984 Dec;58(4):641-8. doi: 10.1111/j.1365-2141.1984.tb06111.x.
2
The use of porcine factor VIII concentrate (Hyate:C) in the treatment of patients with inhibitor antibodies to factor VIII. A multicenter US experience.
Arch Intern Med. 1989 Jun;149(6):1381-5.
3
Use of porcine factor VIII for surgical procedures in hemophilia A patients with inhibitors.
Semin Hematol. 1993 Apr;30(2 Suppl 1):10-21.
4
Comparison of anti-human and anti-porcine factor VIII inhibitor levels in 63 patients with severe haemophilia A. A French Multicentric Study.
Vox Sang. 1993;64(4):210-4. doi: 10.1111/j.1423-0410.1993.tb03057.x.
5
Clinical experience with polyelectrolyte-fractionated porcine factor VIII concentrate in the treatment of hemophiliacs with antibodies to factor VIII.聚电解质分级猪源凝血因子VIII浓缩物治疗血友病伴VIII因子抗体患者的临床经验
Blood. 1984 Jan;63(1):31-41.
6
Experience with highly purified porcine factor VIII in a patient with haemophilia A and a factor VIII inhibitor.
Clin Lab Haematol. 1983;5(1):55-9. doi: 10.1111/j.1365-2257.1983.tb00496.x.
7
Inhibitor antibodies to factor VIII and factor IX: management.针对凝血因子VIII和凝血因子IX的抑制性抗体:管理
Semin Thromb Hemost. 2000;26(2):179-88. doi: 10.1055/s-2000-9821.
8
The use of polyelectrolyte-fractionated porcine factor VIII in the treatment of a spontaneously acquired inhibitor to factor VIII.使用经聚电解质分级分离的猪源因子VIII治疗自发性获得性因子VIII抑制物。
Thromb Res. 1989 Jul 1;55(1):51-6. doi: 10.1016/0049-3848(89)90455-6.
9
[Outcome of hemorrhage treatment in children with hemophilia A related to factor VIII inhibitor].
Acta Haematol Pol. 1994;25(4):349-54.
10
Porcine factor VIII provides clinical benefit to patients with high levels of inhibitors to human and porcine factor VIII.
Haemophilia. 2000 Sep;6(5):482-5. doi: 10.1046/j.1365-2516.2000.00403.x.

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Bioengineered coagulation factor VIII enables long-term correction of murine hemophilia A following liver-directed adeno-associated viral vector delivery.经生物工程改造的凝血因子 VIII 可通过肝靶向腺相关病毒载体递送来实现对小鼠血友病 A 的长期纠正。
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