In the last three decades there have been dramatic improvements in the availability and quality of treatment for people with
inherited coagulation disorders. Indeed, the improvement of methods of purification and viral inactivation for plasma-derived
coagulation factor concentrates first and then the development of products utilizing
recombinant DNA technology have greatly improved the life expectancy of hemophiliacs, which has progressively become similar to that of males in the general population. Nowadays, the most frequent complication of factor replacement
therapy for
hemophilia is the development of inhibitors. However, no studies so far have systematically analysed the type and incidence of other adverse reactions following the administration of
coagulation factor concentrates. The aim of this systematic review was to screen the published literature data to evaluate the types and frequencies of non-thrombotic-, non-inhibitor-associated adverse reactions to
coagulation factor concentrates in patients with
hemophilia A,
hemophilia B and
von Willebrand's disease. On behalf the European
Haemophilia Safety Surveillance System (EUHASS), a systematic review of the prospective studies published in the last 20 years was performed using electronic databases and article references. Both severe and mild adverse events following infusion of
coagulation factor concentrates are relatively rare in patients with
inherited coagulation disorders; the most common events are of an allergic type. There are no differences in the rate of adverse events caused by plasma-derived or recombinant products. On the whole, these data confirm the high degree of safety of the products currently used for replacement
therapy.