Spontaneous reversal of acquired autoimmune dysfibrinogenemia probably due to an antiidiotypic antibody directed to an interspecies cross-reactive idiotype expressed on antifibrinogen antibodies.

Abstract

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 a 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 antiidiotype 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.

Acquired Dysfibrinogenemia Secondary to Multiple Myeloma

“…The production of autoantibodies directed against fibrinogen as a cause of dysfibrinogenemia has been observed in the past [3,4,5,6]. Acquired dysfibrinogenemia occurs mainly as a disorder secondary to liver disease [7,8,9], liver cirrhosis [10, 11], multiple myeloma [12,13,14,15], hepatoma [16, 17], paraneoplastic syndrome [18] or digital ischemia and gangrene [19].…”

“…Acquired dysfibrinogenemia occurs mainly as a disorder secondary to liver disease [7,8,9], liver cirrhosis [10, 11], multiple myeloma [12,13,14,15], hepatoma [16, 17], paraneoplastic syndrome [18] or digital ischemia and gangrene [19]. It was reported that acquired dysfibrinogenemia may be associated with drug usage [20, 21], antifibrinogen antibody presence [4] or allogeneic bone marrow transplantation [22]. …”

A spontaneous hybridoma producing autoanti‐idiotypic antibodies that recognizea Vx−associated idiotope in mercury‐induced autoimmunity

“…Idiotypic interactions have been shown to play a role in the regulation of various autoimmune disorders [15][16][17][18][19][20][21][22] according to the prediction of Jerne's network theory [5]. Involvement of autoanti-Id antibodies in the regulation of autoimmunity [15, 161 has been suggested in systemic lupus erythematosus [17] and in lupus-prone mice [MI, in myasthenia gravis [19], in Hashimoto's and Grave's diseases [20] and in patients with anti-VIII-C [21] or antifibrinogen [22] autoantibodies.…”

“…Involvement of autoanti-Id antibodies in the regulation of autoimmunity [15, 161 has been suggested in systemic lupus erythematosus [17] and in lupus-prone mice [MI, in myasthenia gravis [19], in Hashimoto's and Grave's diseases [20] and in patients with anti-VIII-C [21] or antifibrinogen [22] autoantibodies.…”

Acquired dysfibrinogenemia caused by monoclonal production of immunoglobulin light chain

“…13 There are also examples of anti-fibrinogen antibodies developing spontaneously for no determined reason. [14][15][16] Anti-fibrinogen alloantibodies are equally rare, but there have been reports of their appearance in afibrinogenemic patients who have received fibrinogen replacement therapy. [17][18][19] Another patient developed alloantibodies against fibrinogen, thrombin and factor V after receiving bovine topical thrombin as a hemostatic agent.…”