engleski

Association of Fibrinolytic Parameters with Coagulation Activity and Fibrin Clot Permeability in Patients with Hemophilia A

Hemophilia A has been considered as a bleeding disorder that is not only a consequence of the defect in the coagulation system, but also of the impaired down-regulation of the fibrinolytic system. To examine correlation between activities and/or concentrations of most important fibrinolytic parameters with overall hemostasis potential (OHP) and fibrin clot permeability (FCP) in hemophilia A patients. Activities of FXIII, plasminogen, plasmin inhibitor, plasminogen activator inhibitor–1 (PAI-1) (Siemens Healthcare Diagnostics, Germany) and thrombin-activatable fibrinolysis inhibitor (TAFI) (Diagnostica Stago, France), as well as activated/inactivated TAFI antigen (TAFIa/ai) (Diagnostica Stago) and prothrombin fragment F1+2 concentrations (PF1+2) (Siemens) were tested in plasma samples of 63 hemophilia A patients (30 severe, 33 non-severe). OHP method, based on repeated spectrophotometric registration of fibrin-aggregation in plasma, after addition of small amounts of exogenous thrombin, tissue plasminogen activator and calcium, gave beside OHP parameter (area under the fibrin aggregation curve) 3 supplementary parameters: overall coagulation potential (OCP), overall fibrinolytic potential (OFP) and clot lysis time (CLT). Permeability coefficients (Ks), providing information on fibrin network porosity, were obtained by FCP method, flow measurement technique along with visualisation by electron microscopy scanning. Among fibrinolytic and OHP parameters significant but weak correlation (P< ; 0.05) was found only for OCP with TAFI and PF1+2 (r=0.252 and 0.266, respectively). Regarding FCP, significant correlation of Ks was found with FXIII, plasmin inhibitor and PF1+2 (r=-0.466, - 0.432 and -0.599, respectively). There was no correlation between Ks and OHP parameters. Obtained correlation between fibrinolytic parameters and parameters that assess coagulation activity and fibrin network porosity confirmed the association between these two processes in patients with hemophilia A.

Hemophilia A ; fibrinolytic system ; overall hemostasis potential (OHP) ; fibrin clot permeability (FCP)

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