Factor XIII deficiency – where we can save

Factor XIII deficiency is one of the rare bleeding disorders with an approximate prevalence about 1 per 3 million. A characteristic feature of this deficiency is the high frequency of severe bleeding, including intracranial hemorrhages. This coagulopathy is not detected using a standard coagulation tests, measuring of factor XIII activity is required. The world practice of treating this hemorrhagic condition is the use of plasma and recombinant concentrate. These drugs are not registered in the territory of the Russian Federation. In our country, cryoprecipitate is used. This article describes the clinical case, diagnosis and unsatisfactory results of cryoprecipitate therapy in a patient with diagnosis of factor XIII deficiency. Prompt registration of the XIII factor concentrates is required.