Peake I R, Bloom A L, Giddings J C, Ludlam C A
Br J Haematol. 1979 Jun;42(2):269-81. doi: 10.1111/j.1365-2141.1979.tb01131.x.
An immunoradiometric assay (IRMA) has been developed based on the inhibitor which arose in a polytransfused severe haemophiliac. The two-site IRMA measures antigens closely associated with the procoagulant parts of the factor VIII complex, which are termed FVIIC antigens or FVIIICAG. FVIIICAG was present in normal plasma and also, at a slightly lower concentration, in normal serum. In 37 patients with haemophilia A, 36 had FVIIICAG levels of less than 10% of the normal plasma pool. In patients with von Willebrand's disease the levels of FVIIIC and FVIIICAG were in good agreement, both before and after treatment with cryoprecipitate or DDAVP. FVIIICAG was relatively stable in plasma at 37 degrees C and could also be detected in cord and fetal serum. The assay is of potential value for detecting reduced levels of factor VIII, for carrier detection and for the prenatal diagnosis of haemophilia.
基于一名多次输血的严重血友病患者体内产生的抑制剂,开发了一种免疫放射分析(IRMA)方法。双位点IRMA可检测与因子VIII复合物促凝部分密切相关的抗原,这些抗原被称为FVIIC抗原或FVIIICAG。FVIIICAG存在于正常血浆中,在正常血清中的浓度略低。在37例甲型血友病患者中,36例的FVIIICAG水平低于正常血浆池的10%。在血管性血友病患者中,使用冷沉淀或去氨加压素治疗前后,FVIIIC和FVIIICAG水平均高度一致。FVIIICAG在37℃的血浆中相对稳定,在脐带血和胎儿血清中也可检测到。该检测方法在检测因子VIII水平降低、携带者检测和血友病产前诊断方面具有潜在价值。