Factor XIII (fibrin stabilizing factor [FSF]) is a coagulation factor necessary for the formation of stable fibrin clots. In the presence of calcium ions1,2 and thrombin,3 factor XIII promotes the formation of ε(γglutamyl)lysine covalent bonds between fibrin monomers.4,5 Such bonds render fibrin clots stable in 5M urea or dilute solutions of monochloroacetic acid.
The first patient with a congenital deficiency of factor XIII was described by Duckert et al in 1960.6 Subsequently, many other cases have been reported.7 Absence of factor XIII may result in severe bleeding. Umbilical cord bleeding is usually the earliest manifestation and, characteristically, patients bruise easily and experience excessive bleeding following trauma. Wound healing may be delayed, and disfiguring scar formation has been reported.6 Hemarthroses also occur but are rare.8
Diminished factor XIII activity has been reported in patients with liver disease, leukemia, lymphoma, and some other diseases.9-11