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凝血因子VIII抑制剂的促凝特异性

Procoagulant specificity of factor VIII inhibitor.

作者信息

Yang H C, Kuzur M

出版信息

Br J Haematol. 1977 Nov;37(3):429-33. doi: 10.1111/j.1365-2141.1977.tb01014.x.

DOI:10.1111/j.1365-2141.1977.tb01014.x
PMID:304738
Abstract

Nine haemophilia A patients with an inhibitor to factor VIII procoagulant and eight without an inhibitor were studied for the presence of an inhibitor to von Willebrand factor (vWf) in a quantitative ristocetin-induced platelet aggregation system. The mean vWf, factor VIII related antigen (FVIII Ag) and vWf:FVIII Ag ratio were not significantly different in the two groups (P greater than 0.6). The inhibitor plasmas did not reduce the wWf level in normal plasma after a 2 h incubation. The factor VIII inhibitor is highly specific for the procoagulant function of the factor VIII complex.

摘要

在定量的瑞斯托霉素诱导的血小板聚集系统中,对9例有抗凝血因子Ⅷ抑制剂的甲型血友病患者和8例无抑制剂的患者进行了血管性血友病因子(vWf)抑制剂检测。两组患者的平均vWf、因子Ⅷ相关抗原(FVIII Ag)和vWf:FVIII Ag比值无显著差异(P>0.6)。抑制剂血浆在孵育2小时后未降低正常血浆中的vWf水平。凝血因子Ⅷ抑制剂对凝血因子Ⅷ复合物的促凝血功能具有高度特异性。

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1
Procoagulant specificity of factor VIII inhibitor.凝血因子VIII抑制剂的促凝特异性
Br J Haematol. 1977 Nov;37(3):429-33. doi: 10.1111/j.1365-2141.1977.tb01014.x.
2
IgA inhibitor to factor VIII/von Willebrand factor.
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3
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4
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The combined use of monoclonal antibody-based enzyme-linked immunosorbent assays (ELISA) for factor VIII antigen (VIII:Ag) and von Willebrand factor antigen (vWF:Ag) for the detection of carriers of haemophilia A.基于单克隆抗体的酶联免疫吸附测定法(ELISA)联合检测因子VIII抗原(VIII:Ag)和血管性血友病因子抗原(vWF:Ag)以检测甲型血友病携带者。
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Autoantibody to factor VIII that has less reactivity to factor VIII/von Willebrand factor complex.对VIII因子具有自身抗体,该自身抗体对VIII因子/血管性血友病因子复合物的反应性较低。
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Pseudo-von Willebrand's disease. An intrinsic platelet defect with aggregation by unmodified human factor VIII/von Willebrand factor and enhanced adsorption of its high-molecular-weight multimers.假性血管性血友病。一种血小板内在缺陷,其表现为未修饰的人凝血因子VIII/血管性血友病因子可引起血小板聚集,且其高分子量多聚体的吸附增强。
N Engl J Med. 1982 Feb 11;306(6):326-33. doi: 10.1056/NEJM198202113060603.