Prophylactic Program in fxiii Deficient Patients From Iran

Prophylaxis is the management strategy in FXIII deficient patients. Life-long prophylactic therapy by 10–20 U/kg FXIII (every 4–6 weeks) is recommended in patients with severe FXIII deficiency to prevent life-threatening spontaneous bleeding. In this study we evaluated of the efficacy and safety of prophylaxis program in a large study group consisting of 182 FXIII deficient Iranian patients. Material and methods: A total of 182 FXIII deficient patients (98 male and 84 female, mean age 26 years old) from south and south-east Iran were selected as a study group. Continual prophylaxis was done in all patients for periods of 6–168 months (mean 66.6 months). The patients received Fibrogamin-P (Dade Behring, Marburg, Germany) 10–20 U/kg every 4–6 weeks and before availability of this concentrate in Iran the prophylaxis was done by FFP 15–20 ml/kg every 20–30 days. All of patients were followed up for any bleeding diathesis and adverse effects during the prophylaxis period. Results: A total of 189 bleeding episodes including epistaxis, gum bleeding, bleeding after dental extraction, hemarthrosis, hematoma, hematuria, wound bleeding, menorrhagia and abortion were detected in 182 patients after prophylaxis program. Life-threatening intracranial bleeding was found in 34 patients before starting prophylaxis; however, there were no reports after prophylaxis. The bleeding episodes and bleeding score were significantly decreased after prophylaxis in compare to before starting prophylaxis (P<0.05) Discussion: The prophylaxis of FXIII deficiency by FXIII concentrate is seems clinical significance in prevention of spontaneous and life-threatening bleeding and also abortion in affected women. Also it is recommended to start prophylaxis in patients as soon as possible for prevention of life-threatening bleeding episodes.

No relevant conflicts of interest to declare.