Thrombosis research
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Thrombosis research · Mar 1987
Case ReportsFibrinogen Barcelona I. Congenital dysfibrinogenemia characterized by defective release of fibrinopeptide A and fibrinogen degradation products.
A congenital dysfibrinogenemia, fibrinogen Barcelona I, was detected in a 28 year-old woman with no prior history of bleeding. The thrombin induced clotting of plasma and purified fibrinogen was much prolonged. Fibrin monomer aggregation was impaired. ⋯ Plasmin digestion of two fibrinogens showed identical patterns in SDS-PAGE as regards X fragment formation. The kinetics of fibrinogen degradation showed a decrease in the formation rate of D and E fragments. The fact that the patient was in threat of abortion and developing a haemorrhagic syndrome may indicate that the defect in the fibrinogen was important in the pathogenesis of haemorrhage in this patient.
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In order to evaluate the influence of heat treatment (68 degrees C for 24 or 72 hours) on the essential components of antihaemophilic cryoprecipitate, i.e. factor VIII coagulant activity (VIII:C), von Willebrand factor (VIIIR:Ag and VIIIR:RCF) and fibrinogen, ordinary lyophilized cryoprecipitate was compared to heat treated, aminoacid-enriched specimens. The median reduction in factors VIII:C, VIIIR:Ag, VIIIR:RCF and fibrinogen during lyophilization of ordinary cryoprecipitate was 26 per cent, 11 per cent, 1 per cent and 8.5 per cent, respectively. Heat treatment of such cryoprecipitate resulted in 85 to 98.5 per cent reduction in these parameters, while the reduction following lyophilization and heat treatment (24 hours) of aminoacid-containing preparations was not significantly different from non-heated, ordinary cryoprecipitate. ⋯ Enrichment with aminoacids, however, made the heat treated cryoprecipitate fully soluble, but the content of these vials were slightly slower in dissolving than non-heated preparations. Ultracentrifugation prior to lyophilization and heating did not improve the solubility. If heat treatment proves to be efficient in inactivating viral agents, we conclude that heated (68 degrees C for 24 hours), aminoacid-enriched cryoprecipitate may be a convenient product for treating haemophilia A, von Willebrand's disease and hypofibrinogenemia.