HEMOPHILIA INHIBITOR PATIENTS: THE USE OF RECOMBINANT FACTOR VIIa
Abstract
Background. Recombinant factor VIIa can induce hemostasis and controls bleeding in surgery in hemophilia inhibitor patients and in other hemostatic disorders, e.g. thrombocytopenia, platelet function disorders, coagulation disorders due to drugs and liver disease, in trauma and surgical patients. We describe the use of rF VIIa in bleeding episodes and surgery in four hemophilia A inhibitor patients.
Patients and methods. We used rF VIIa in 6 bleeding episodes and in 3 surgical procedures (intracerebral and subdural hematoma, hematoma of the sculp) in four hemophilia inhibitor patients. 90 µg/kg b.w. rF VIIa was given every 2 hours for 24–82 hours, then the time interval was gradually changed to 6 hours.The treatement duration depends on the site and severity of bleeding and surgery. The traditional assays are not suitable for monitoring the hemostatic effect of rF VIIa.
Results and discussion. The reason for rF VIIa treatement were inhibitors in hemophilia, necessity for treatement for several days and surgery. In muscle, joint, gastrointestinal bleeding rF VIIa was effective but not in hematuria and thoracic wall bleeding with haemorhagic shock. The surgery itself went smoothly.
Conclusions. rF VIIa controls spontaneous and posttraumatic bleedings, even the longlasting ones in hemophilia A (and B) patients with inhibitors. It prevents bleeding in major surgery. Adult patients should be treated first with standard doses of 90 µg/kg b.w. every 2 hrs for 24–48 hrs. Than time interval is gradually prologed to 6 hrs. The traditional assays are not suitable for monitoring the hemostatic effect of rF VIIa. Close monitoring is required in all patients to determine efficacy and insure safety of treatement.
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References
Hedner U. Dosing with recombinant factor VIIa based on current evidence. Semin Hematol 2004; 41: suppl 1: 35–9.
Oldenburg J, Schroeder J, Brackmann HH et al. Enviromental and genetic factors influencing inhibitor development. Semin Hematol 2004; 41: suppl 1: 1–14.
Tagariello G, DeBiasi E, Gajo GB et al. Recombinant FVIIa (NovoSeven) continuous infusi0n and total hip replacement in patients with haemophilia and high titre of inhibitors to F VIII: experience of two cases. Haemophilia 2000; 6: 581–3.
Hedner U, Bjoern S, Bernvil SS, Tengborn L, Stigendahl L. Clinical experience with human plasma-derived F VIIa in patients with hemophilia A and high titer inhibitors. Haemostas 1989; 19: 335–43.
Kenet G, Lubetsky A, Luboshitz J, Martinowitz U. A new approach to treatement of bleeding episodes in young hemophilia patients: a single bolus megadose of recombinant activated factor V II (NovoSeven). J Thromb Haemostas 2002; 1: 450–55.
Gabriel don A, Carr M, Roberts HR. Monitoring coagulation and the clinical effects of recombinant factor VIIa. Semin Hematol 2004; 41: suppl 1: 20–24.
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