Intracranial hemorrhage (ICH) is one of the most severe and life-threatening manifestations occurring in the patients with
factor XIII (F XIII) deficiency. The aim of this study was to describe the ICH pattern in the patients suffering from F XIII deficiency. In this case series, we investigated 38 patients with severe F XIII deficiency in south of Iran from January to May 2012. ICH pattern, neurologic complications, efficacy of treatment, and incidence of recurrence were reported. The site of ICH was intraparenchymal in 35 patients (92.1 %), subdural in 2 patients (5.2 %), and epidural
hemorrhage in 1 patient (2.6 %). Besides, neurologic complications occurred in 21 patients (55.2 %), including locomotor disability in 8, psychological impairment in 7,
mental disorders in 5, speech impairment in 4, and
visual impairment in 2. Prophylaxis was started with a dose of 10 IU/kg
Fibrogammin every 4-6 weeks for all the patients, except for one. All the patients on prophylaxis showed good response without any episodes of recurrence, except for one. The most frequent site of ICH in our patients was intraparenchymal. It seems that long-term prophylactic treatment with a dose of 10 IU/kg
Fibrogammin could be effective in the prevention of CNS
bleeding in the patients with F XIII deficiency. Moreover, all the patients with severe F XIII deficiency even without severe
bleeding symptoms are recommended to undergo prophylactic treatment.