Spontaneous reversal of acquired autoimmune dysfibrinogenemia probably due to an antiidiotypic antibody directed to an interspecies cross-reactive idiotype expressed on antifibrinogen antibodies.
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.
Ruiz-Arguelles
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A
Department of Immunology, Laboratorios Clinicos de Puebla, Mexico.
D000328:Adult; D000347:Afibrinogenemia / Q000097:blood / Q000276:immunology*; D000818:Animals; D000888:Antibodies, Anti-Idiotypic / Q000235:genetics / Q000302:isolation & purification / Q000502:physiology*; D001323:Autoantibodies / Q000302:isolation & purification / Q000502:physiology*; D001327:Autoimmune Diseases / Q000097:blood / Q000276:immunology*; D003429:Cross Reactions; D005340:Fibrinogen / Q000235:genetics / Q000276:immunology*; D006801:Humans; D007130:Immunoglobulin Idiotypes / Q000235:genetics / Q000276:immunology*; D008297:Male; D011817:Rabbits; D013045:Species Specificity
D000888:Antibodies, Anti-Idiotypic; D001323:Autoantibodies; D007130:Immunoglobulin Idiotypes; D005340:Fibrinogen