Ruiz-Arguelles A
Department of Immunology, Laboratorios Clinicos de Puebla, Mexico.
J Clin Invest. 1988 Sep;82(3):958-63. doi: 10.1172/JCI113704.
A young man with a long history of abnormal bleeding was seen in January 1985. Coagulation tests showed dysfibrinogenemia and an antifibrinogen autoantibody was demonstrable in his serum. This antibody, when purified, was capable of inhibiting the polymerization of normal fibrin monomers, apparently through binding to the alpha fibrinogen chain. 6 mo later the patient was asymptomatic, coagulation tests were normal, and the antifibrinogen autoantibody was barely detectable. At this time, affinity-purified autologous and rabbit antifibrinogen antibodies were capable of absorbing an IgG kappa antibody from the patient's serum, which reacted indistinctly with both autologous and xenogeneic antifibrinogen antibodies in enzyme immunoassays. It has been concluded that the patient's dysfibrinogenemia was the result of an antifibrinogen autoantibody, and that later on an anti-idiotype antibody, which binds an interspecies cross-reactive idiotype expressed on anti-human fibrinogen antibodies, inhibited the production of the antifibrinogen autoantibody which led to the remission of the disorder.
1985年1月接诊了一名有长期异常出血史的年轻男子。凝血检查显示存在异常纤维蛋白原血症，其血清中可检测到抗纤维蛋白原自身抗体。纯化后的这种抗体能够抑制正常纤维蛋白单体的聚合，显然是通过与α纤维蛋白原链结合来实现的。6个月后，患者无症状，凝血检查正常，抗纤维蛋白原自身抗体几乎检测不到。此时，亲和纯化的自体和兔抗纤维蛋白原抗体能够从患者血清中吸收一种IgG κ抗体，该抗体在酶免疫测定中与自体和异种抗纤维蛋白原抗体的反应不明显。得出的结论是，患者的异常纤维蛋白原血症是抗纤维蛋白原自身抗体所致，后来一种抗独特型抗体结合了抗人纤维蛋白原抗体上表达的种间交叉反应独特型，抑制了抗纤维蛋白原自身抗体的产生，从而导致病情缓解。
