Thrombophlebitis Migrans Associated with Circulating Antibodies against Fibrinogen

Abstract

SummaryThe clinical course of a young patient is described who developed a thrombophlebitis migrans associated with pulmonary embolism after a pneumonia. Circulating antibodies against fibrinogen were found, and the possible mechanisms of the formation of these antibodies, as well as the possible role of these antibodies in the development of the thrombophlebitic process are discussed. A combined intravenous therapy with heparin and steroids in high doses was successful in eliminating a further antibody production and clotting.

Idiopathic autoantibody that inhibits fibrin monomer polymerization

“…The formation of antibodies interfering with the release of fibrinopeptide A had been described in a patient with Down's syndrome (Marciniak CG Greenwood, 19 79). and anti-fibrinogen antibodies have been described in two patients with congenital afibrinogenaemia (De Vries et al, 1961: Bronniman, 1 9 54) following fibrinogen transfusions and in one patient with migratory thrombophlebitis with normal fibrinogen levels (Mammen et a/. 1962).…”

Antithrombin-III deficiency in a Dutch family

“…Studies on subjects with an increased tendency to venous thrombosis have pointed to various plasma factors which might indicate or be the cause of hypercoagulability: inhibition of plasminogen activation (Nilsson, Krook, Sternby, Soderberg, and Soderstrom, 1961), antithrombin-III deficiency (Egeberg, 1965;Von Kaulla and Von Kaulla, 1967;Penick, 1969), elevated factor Vlevels (Gaston, 1966), circulating antibodies against fibrinogen (Mammen, Schmidt, and Barnhart, 1967), abnormal fibrinogen (Egeberg, 1967), circulating fibrin monomers (Vreeken, Fedder, Van der Meer, Van Aken, Prakke, and Goote, 1972), and deficiencyofplasminogen activator (Isacson and Nilsson, 1972). In 1965 Egeberg described a Norwegian family with a high incidence of venous thrombosis.…”

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

“…The demonstration of the existence of an autoantibody directed against fibrinogen, which was capable of inhibiting fibrin aggregation during the active phase of the hemorrhagic disease but not while the patient was in clinical remission, strongly suggests that the antifibrinogen autoantibody was the cause of the fibrinogen dysfunction. Other cases of autoimmune dysfibrinogenemia have been reported in the past (12)(13)(14)(15). In some cases the antibody inhibited polymerization of fibrinogen through blocking the cleavage sites for thrombin, while in others the antibody inhibited the aggregation of fibrin monomers after thrombin cleavage.…”

“…In fact, it is presumable that the expression of autoimmune humoral immune responses might be modulated, ita, est, augmented, or depressed, by anti-Id antibodies (6, 9-1 1). The production of autoantibodies directed to fibrinogen as a cause of dysfibrinogenemia has been observed in the past (12)(13)(14)(15). In some instances, these antibodies interfere with fibrinogen clotting by inhibiting the cleavage of fibrinopeptides A and B by thrombin, while in other instances they block the polymerization of fibrin monomers after thrombin cleavage.…”