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[非血友病患者与重度甲型血友病患者中因子VIII抑制剂的比较研究]

[Comparative study of a factor-VIII inhibitor in a non-hemophylic patient and in a patient with hemophylia A gravis].

作者信息

Mayer C, Kalogjera V, Lievre C, Pautler J, Weisel M L, Mayer S

出版信息

Sem Hop. 1977;53(25-28):1511-6.

PMID:19846
Abstract

In this study, we tried to make a comparison between the findings concerning two human strong anti Factor VIII inhibitors. In one case, the strong inhibitor appeared in an old man without any particular antecedant. In the second case, it occurred in a young hemophiliac A. We found in both cases that the inhibitor was a immunoglobulin G with light chain lambda. Its activity site is on the fragment AB. These anticoagulants have a inhibitory action against only the Factor VIII procoagulant fraction and have exclusively neutralising properties. The inhibitor present in the non hemophiliac patient has particularly great resistance to temperature and pH variations.

摘要

在本研究中,我们试图对关于两种人类强效抗凝血因子VIII抑制剂的研究结果进行比较。在一个病例中,强效抑制剂出现在一位无任何特殊既往史的老年男性身上。在第二个病例中,它出现在一名年轻的甲型血友病患者身上。我们在这两个病例中均发现,该抑制剂是一种带有λ轻链的免疫球蛋白G。其活性位点位于AB片段上。这些抗凝剂仅对凝血因子VIII促凝部分具有抑制作用,且仅具有中和特性。非血友病患者体内的抑制剂对温度和pH变化具有特别强的耐受性。

相似文献

1
[Comparative study of a factor-VIII inhibitor in a non-hemophylic patient and in a patient with hemophylia A gravis].[非血友病患者与重度甲型血友病患者中因子VIII抑制剂的比较研究]
Sem Hop. 1977;53(25-28):1511-6.
2
[Development of factor VIII inhibitor in three non-hemophiliac patients].[三名非血友病患者中因子 VIII 抑制剂的发生情况]
Rinsho Ketsueki. 2000 Nov;41(11):1195-200.
3
Immunochemical characterization of factor VIII inhibitors.凝血因子 VIII 抑制剂的免疫化学特性分析
Prog Clin Biol Res. 1984;150:73-85.
4
[Successful induction of immune tolerance and novel hemostatic effects in a hemophilia A with high-responder inhibitor by regular infusions of factor VIII].[通过定期输注凝血因子 VIII 在高反应性抑制物的甲型血友病患者中成功诱导免疫耐受及产生新型止血效果]
Rinsho Ketsueki. 1996 Nov;37(11):1303-8.
5
[Hemorrhagic syndrome associated with a factor VIII:C inhibitor in a patient with lymphoma].[淋巴瘤患者伴Ⅷ因子:C抑制物的出血综合征]
Med Clin (Barc). 1989 Jun 3;93(1):23-5.
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The immunological characterization of human antibodies to factor VIII isolated by immuno-affinity chromatography.通过免疫亲和色谱法分离的人抗凝血因子 VIII 抗体的免疫学特性分析。
Thromb Haemost. 1981 Feb 23;45(1):60-4.
7
[Factor VIII epitopes recognized by inhibitors in hemophiliacs].
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8
An inhibitor to factor VIII:C in a patient with possible combined haemophilia A and von Willebrand's disease.一名可能同时患有甲型血友病和血管性血友病患者体内的凝血因子VIII:C抑制剂。
Thromb Haemost. 1986 Apr 30;55(2):158-61.
9
Prevalence of the intron 22 inversion of the factor VIII gene and inhibitor development in Polish patients with severe hemophilia A.波兰重度A型血友病患者中凝血因子VIII基因内含子22倒位的患病率及抑制物的产生
Arch Immunol Ther Exp (Warsz). 2005 Jul-Aug;53(4):352-6.
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[Acquired hemophilia. Apropos of a case report with a fatal course].[获得性血友病。关于一例致死病程的病例报告]
Schweiz Med Wochenschr. 1988 Oct 22;118(42):1542-5.