title: Non-thrombotic-, non-inhibitor-associated adverse reactions to coagulation factor concentrates for treatment of patients with hemophilia and von Willebrand's disease: a systematic review of prospective studies.
abstract: 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.
authors.affiliation: Department of Transfusion Medicine and Hematology, Carlo Poma Hospital, Mantova, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Franchini
authors.forename: M
authors.initials: M
authors.lastname: Makris
authors.forename: E
authors.initials: E
authors.lastname: Santagostino
authors.forename: A
authors.initials: A
authors.lastname: Coppola
authors.forename: P M
authors.initials: PM
authors.lastname: Mannucci
mesh_terms: D003029:Coagulants / Q000009:adverse effects*; D005164:Factor IX / Q000009:adverse effects*; D005169:Factor VIII / Q000009:adverse effects*; D006467:Hemophilia A / Q000188:drug therapy*; D002836:Hemophilia B / Q000188:drug therapy*; D006801:Humans; D008297:Male; D011446:Prospective Studies; D011994:Recombinant Proteins / Q000009:adverse effects; D014842:von Willebrand Diseases / Q000188:drug therapy*
chemical_list: D003029:Coagulants; D011994:Recombinant Proteins; D005169:Factor VIII; D005164:Factor IX