Factor XIII deficiency

Factor XIII deficiency is an exceedingly rare bleeding disorder that causes a severe bleeding tendency. Its incidence is given as one in a million to one in five million people, with higher incidence in areas where consanguineous marriage is common, such as Iran, which has the highest global incidence. Most cases are due to mutations in the gene for the A subunit, located on chromosome 6p25-p24, and the condition is inherited in an autosomal recessive fashion. Deficiency leads to defective cross-linking of fibrin and vulnerability to late re-bleeds, with hemophilia-like bleeding such as hemarthroses and deep tissue bleeding. Recombinant A subunit is becoming a therapeutic option.