Successful delivery in patients with FXIII deficiency receiving prophylaxis: report of 17 cases in Iran.

To prevent pregnancy loss in women with severe FXIII deficiency, prophylactic replacement therapy with a source of FXIII throughout pregnancy is essential. The aim of this study was to evaluate the bleeding score and rate of successful deliveries in FXIII-deficient pregnant Iranian women receiving regular prophylaxis. Seventeen FXIII-deficient women 18-35 years old (mean 24 years) were enrolled in the study. All patients except one had a history of at least one miscarriage. Patients received regular prophylaxis with 10 IU kg(-1) FXIII concentrate every 4 weeks before pregnancy and every 2 weeks during pregnancy for a period of 24-62 months. All bleeding episodes were recorded, and the bleeding score was determined on a standard form before and after the start of prophylaxis. After starting prophylaxis, monochloroacetic acid tests and 5 m urea tests were normal in all patients, and the bleeding score significantly decreased from 11-16 (mean 12 ± 1.5) to 23 (mean 2.2 ± 0.4) (P < 0.001). Thirteen minor bleeding episodes occurred during prophylaxis. All patients successfully delivered at 36 weeks' gestation and there were no significant coagulation complications during or after delivery. In this study, successful pregnancy maintenance and delivery were achieved in Iranian women with severe FXIII deficiency. Precise detection and diagnosis of this condition in women with coagulation disorders is essential to enable implementation of appropriate prophylaxis to prevent pregnancy loss.