Razi Journal of Medical Sciences

Factor XIII deficiency is a bleeding disorder that its prevalence in the general population is about 1 in 2 million people around the world. In Iran, the high rate of consanguineous marriages lead to high rate of disorder with 473 factor XIII deficient patients that is about 12 times higher than the global Prevalence of disorder. The study, is a comprehensive review of all aspects of factor XIII deficiency in Iran. The distribution of the disease in different parts of Iran shows that the province is not only the largest number of patients with factor XIII deficiency in Iran, but also the world's highest incidence of the disease has been reported in this area. In Iranian patients, bleeding from the umbilical cord ,hematoma, prolonged bleeding are the most common clinical presentations. Several disease-causing mutation in Iranian patients with the most common mutation factor XIII deficiency that Trp187Arg southeast of Iran. Traditionally, patients with factor XIII deficiency treated with fresh frozen plasma or cryoprecipitate in Iran until 2009, that factor XIII concentrates for the treatment of patients became available. Several studies on the efficacy and safety of prophylactic regimes in different situations with valuable findings have been evaluated.