Algiman M, Dietrich G, Nydegger U E, Boieldieu D, Sultan Y, Kazatchkine M D
Laboratoire d'Hémostase, Hôpital Cochin, Paris, France.
Proc Natl Acad Sci U S A. 1992 May 1;89(9):3795-9. doi: 10.1073/pnas.89.9.3795.
Spontaneous inhibitors of factor VIII (FVIII) are pathogenic IgG autoantibodies of restricted isotypic heterogeneity found in the plasma of patients presenting with bleeding episodes and low levels of FVIII. We now report the presence of a natural FVIII-neutralizing activity in 85 of 500 plasma samples (17%) from healthy donors. FVIII-inhibitory activity was present in F(ab')2 fragments of purified IgG and was dose-dependent. The titer of anti-FVIII antibodies in normal plasma ranged between 0.4 (threshold of detection) and 2.0 Bethesda units. Anti-FVIII IgG was also detected in normal plasma by using an ELISA. Anti-FVIII antibodies from healthy individuals did not exhibit restricted isotypic heterogeneity. Mean levels of FVIII activity did not differ significantly between individuals with and without detectable anti-FVIII antibodies in plasma. Natural anti-FVIII IgG inhibited FVIII activity in pools of normal plasma and in plasma of certain donors in the pool but did not inhibit FVIII activity in autologous plasma. These observations demonstrate that polyclonal IgG antibodies against procoagulant FVIII are present in healthy individuals. The antibodies are natural IgG autoantibodies and/or antibodies directed against epitopes associated with a so far unidentified allotypic polymorphism of the human FVIII molecule.
因子VIII(FVIII)的自发性抑制剂是在出现出血发作且FVIII水平较低的患者血浆中发现的具有受限同种型异质性的致病性IgG自身抗体。我们现在报告,在500份来自健康供者的血浆样本中有85份(17%)存在天然FVIII中和活性。FVIII抑制活性存在于纯化IgG的F(ab')2片段中,且呈剂量依赖性。正常血浆中抗FVIII抗体的滴度介于0.4(检测阈值)至2.0贝塞斯达单位之间。通过酶联免疫吸附测定(ELISA)也在正常血浆中检测到了抗FVIII IgG。来自健康个体的抗FVIII抗体未表现出受限的同种型异质性。血浆中可检测到抗FVIII抗体的个体与未检测到的个体之间,FVIII活性的平均水平无显著差异。天然抗FVIII IgG抑制正常血浆池以及该血浆池中某些供者血浆中的FVIII活性,但不抑制自身血浆中的FVIII活性。这些观察结果表明,健康个体中存在针对促凝血FVIII的多克隆IgG抗体。这些抗体是天然IgG自身抗体和/或针对与人FVIII分子迄今未确定的同种异型多态性相关表位的抗体。