Recombinant Factor VIIa and Activated Prothrombin-Complex Concentrate Administration in the Management of Bleeding, Coagulopathy and Intractable Coagulopathy in Pediatric Patients Undergoing Invasive Medical Procedures or Surgery

Coagulation factor VII (FVII) is a vitamin K dependent glycoprotein of the extrinsic pathway and initiates coagulation by binding to tissue factor. Activated prothrombin-complex concentrate ([APCC] FEIBA), an activated prothrombin complex concentrate that is plasma-derived, has also been demonstrated to bypass the need for FVIII or factor IX, resulting in hemostasis. Both APCC and recombinant factor VII ([rFVIIa] NovoSeven) are used for the treatment of bleeding episodes in patients with hemophilia A or B with inhibitors to Factor VIII or IX and in those with congenital Factor VII deficiency. The aim of the present study was to evaluate the clinical outcomes of APCC or rFVIIa therapy in pediatric patients (that bleeding or coagulopathy) undergoing invasive medical procedures or surgery. Twenty four patients hospitalized in pediatric clinics for intractable bleeding disorders and coagulopathies. The drugs were administered in a total of 35 episodes (25 episodes for APCC and 10 episodes for rFVIIa). Total blood count, blood biochemistry, coagulation tests and appropriate radiological assessments were performed in each patient. Survival times were calculated for each patient. The mean values of prothrombin time (PT) was 29.4±16.5 second, partial thromboplastin time (PTT) was 70.2±43.8 second and fibrinogen level was 247.8±130.2 mg/dl. The mean values after the administration of APCC or rFVIIa were 16.6±7.2 seconds, 41.2±16.0 seconds and 290.3±146.9 mg/dl, PT, PTT and fibrinogen level, respectively. The improvements in the corresponding values after therapy were statistically significant. APCC and rFVIIa can be used to prevent coagulopathy and cessation bleedings. Thus, these concentrates may be effective in reducing the incidence of thrombocytopenia and coagulopathy.