Notfallbehandlung von Blutungskomplikationen bei Hemmkörper-Hämophilie mit aktivierten Prothrombinkomplex-Konzentraten

Abstract

Severe rectal bleeding in a 6-year-old boy with haemophilia A and factor VIII inhibitors could not be stopped with factor VIII concentrates. But a good effect was achieved with activated prothrombin complex concentrates (fraction FEIBA), given over eight days. Amaurosis occurred as a complication after injection of the first dose, but disappeared completely within several minutes. Tests revealed accelerated intravascular coagulation with increased fibrin monomers and fibrin/fibrinogen degradation products.

Failure of factor VIII inhibitor bypassing activity (Feiba) to secure haemostasis in haemophilic patients with antibodies.

“…On the credit side, and as confirmed in the present study, untoward side-effects are few although there has been a report of disseminated intravascular coagulation (Stenbjerg and Jorgensen, 1977a), resistance to Feiba (Stenbjerg and Jorgensen, 1977b), and one disturbing case of transient amaurosis (Rasche et al, 1977). Anamnestic response of factor VIII antibody is less likely to occur than with factor VIII concentrate, and the treatment is certainly much less expensive.…”

Effect of Treatment with Activated Prothrombin Complex Concentrate (FEIBA) on Factor VIII-Antibody Level

“…Activated prothrombin complex preparations have become a new and promising therapeutic tool in the management of haemophiliacs with antibodies to factor VIII (Penner and Kelly 1975). Originally, these concentrates were applied mainly in patients with lifethreatening haemorrhages which could not be controlled by factor VIII treatment and plasmapheresis (Kurczynski and Penner 1974, Goodnight et al 1976, Sonoda et al 1976, Rasche 1977, Schimpf and Zimmermann 1977.…”