ABSTRACT
The standard modality of administration of rFVIIa to patients with FVIII and FIX inhibitors is the intermittent infusion every 2 to 6 hours. No untoward local or systemic effects have been reported; laboratory data of activation of coagulation were reported in the presence of coexistent problems (sepsis, septic shock) or with high doses. We treated four patients with FVIII inhibitor with rFVIIa administered by continuous infusion by a central vein catheter, monitoring the signs of systemic activation of the hemostatic system. The F(1+2) prothrombin fragments and the D-dimer increased after the bolus, and remained above the baseline values throughout the treatment period. These variations observed during the infusion period were not accompanied by clinical events.
Subject(s)
Blood Coagulation/drug effects , Factor VIII/immunology , Factor VII/administration & dosage , Fibrinolysis/drug effects , Adult , Aged , Blood Coagulation/physiology , Catheterization, Central Venous , Factor VII/adverse effects , Factor VII/metabolism , Factor VII/pharmacology , Factor VIII/antagonists & inhibitors , Factor VIIa , Female , Fibrin Fibrinogen Degradation Products/metabolism , Fibrinogen/metabolism , Fibrinolysis/physiology , Hematoma/chemically induced , Hematoma/drug therapy , Hemophilia A/complications , Hemophilia A/drug therapy , Humans , Infusions, Intravenous/methods , Isoantibodies/blood , Male , Middle Aged , Peptide Fragments/blood , Peptide Fragments/metabolism , Platelet Count , Prothrombin/metabolism , Recombinant Proteins/administration & dosage , Recombinant Proteins/adverse effects , Recombinant Proteins/pharmacologyABSTRACT
A patient with severe haemophilia A underwent orthotopic liver transplantation because of changes correlated to end-stage liver cirrhosis due to hepatitis B, C and D infection. Replacement therapy was carried out for 4 days and the clinical course was uneventful. At the time of reporting the patient has a normal working life. FVIII plasma concentration is normal. The indirect hyperbilirubinaemia may be related to the Gilbert's anomaly of the donor.
