Genetic Background of Inherited Factor XIII-A Subunit Deficiency: Review of the Literature and Description of Two New Cases

We read with great interest the paper by Dorgalaleh et al,[1] in this journal, who described inherited factor XIII (FXIII) deficiency and the molecular analysis of this disorder in the Iranian population. Inherited FXIII deficiency is a rare bleeding disorder affecting approximately one person per 1 to 3 million.[2] In areas with an advanced incidence of consanguineous marriages, the prevalence may be higher. The previous authors reported estimated prevalence in the Iranian population as 12-fold higher.[1] An autosomal recessive inheritance pattern is typical; thus, homozygous, or compound heterozygous patients are affected. Heterozygous patients usually do not manifest with bleeding episodes. However, there are reports on heterozygous carriers of FXIII deficiency with prolonged or massive bleeding in stressful situations like minor trauma, pregnancy, or surgery.[3]