Search

Factor XIII (FXIII) plays an important role in the field of blood coagulation. In the last decade, both congenital and acquired deficiencies have been investigated in clinical studies. FXIII is a versatile enzyme that leads to a covalent cross-linking of fibrin fibrils at the end of the clotting cascade and supports platelet adhesion to the damaged sub-endothelium with the result of a mechanically stable clot.Symptoms of FXIII deficiencies vary within a broad spectrum from superficial skin bleeding episodes to severe, sometimes life threatening hemorrhage, requiring prophylactic or therapeutic replacement therapy.Since 1993 purified plasma-derived FXIII concentrate has been available in Germany, large parts of Europe and in the USA and Canada. The administration is conducted intravenously, and FXIII is immediately available in the plasma. The dosage should be determined by measuring actual plasma FXIII-activity. Repetitive application is possible, especially with regard to the mean half-time of 7.9 days.Administration is considered to be safe and effective, but there are some case reports, as with other coagulation factors, describing the appearance of inhibitory antibodies.This summary seeks to provide an insight into the principle pharmacokinetic and pharmacodynamic characteristics of plasma-derived FXIII concentrate, reviewing the current literature. For detailed use in clinical settings, the application of FXIII concentrate or substitution therapy with fresh frozen plasma, we therefore refer to current guidelines and significant studies that have been recently published.