Factor XIII or "
fibrin-stabilizing factor," is a
transglutaminase circulates in the blood circulation as a hetero tetramer with two catalytic A subunits and two carrier B subunits. This important
coagulation factor has a crucial role in clotting cascade and produces strong covalent bonds between soluble formed
fibrin monomers during coagulation. This stable cross linked
fibrin strands are resistanttodegradationby thefibrinolyticsystem that enablesthe bodyto stoppotential
bleeding episodes. In the absence or severe decrease of
factor XIII, although the clot is formed, but is rapidly degraded by the fibrinolytic system, and delayed bleedingoccurs.Factor XIII deficiency is an extremely rare
bleeding disorder with estimated incidence of 1/2-3000, 000 in the general population. Presumptive diagnosis of
factor XIII deficiency was by clot solubility test in 5M
urea or 1%
monochloroacetic acid environments. In patients with abnormal screening clot solubility test, the disease can be confirmedbymore specifictestssuch as quantitative
factor XIII activity assay andFXIIIAgassay.After diagnosis of disease all patients with severe
factor XIII deficiency(<1 U/dl) shouldreceive prophylactic substitution therapywith fresh frozen plasma (FFP) and cryoprecipitate as traditional choices or purified concentrateof
blood coagulation factor XIII (
Fibrogammin P) inorder to control severe and life-threatening clinical complications of
factor XIII deficiency.