An Abnormal Fibrinogen (Copenhagen) Associated with Severe Thromboembolic Disease, but with Normal Adsorption of Plasminogen

An autosomally inherited qualitative fibrinogen (F) defect is presented. The abnormal F was detected in the plasma of a 54 year old woman with severe arterial thrombotic disease. A decreased rate of fibrin formation of plasma, or purified F by thrombin or ancrod (Arvin (R ) ) was demonstrated. The plasma F concentration was normal, when estimated by clottability or immunologic technique. No F related material was present in the serum. The purified abnormal F was indistinguishable from normal F by Immunoelectrophoresis or SDS Polyacrylamide gel electrophoresis. The major defects detected were an abnormality of polymerization of fibrin monomers and a decreased rate of release of fibrinopeptide A. To evaluate a possible abnormality of the binding of plasminogen (P) to the abnormal fibrin, we examined the adsorption of partially degraded P (Lys-P), which has a higher affinity for fibrin than Glu-P. The adsorption was normal, but the study might be useful in the evaluation of dysfibrinogenemia associated with venous thrombosis.