Successful Delivery of Patients with FXIII Deficiency on Prophylaxis in Iran

Coagulation factor XIII (FXIII) deficiency is a rare bleeding disorder with a general frequency of 1 in 2 million populations. Life-long bleeding tendencies, umbilical bleeding, defective wound healing and habitual abortions are the most important clinical problems in this patients.For prevention of abortion and pregnancy loss in severe FXIII-deficient women, replacement and prophylaxis with a source of FXIII throughout pregnancy is essential.The acceptance level of plasma FXIII level for successful delivery is above 10% as a result the administration of FXIII concentrate 250 IU weekly until 22 weeks gestation and 500 IU from 23 weeks is recommended to maintain this plasma level. The aim of this study was to evaluate the bleeding score and rate of successful delivery in Iranian FXIII deficient pregnant women on regular prophylaxis.

A total of 17 FXIII deficient Iranian women with age range 18–35 years old (mean 24 years old) were enrolled in this study. The first record bleeding episodes were cord bleeding in 10 patients, echymosis in 4 patients, hemarthrosis in 1 patient, hematoma in 1 patient and CNS bleeding in 1 patient. All patients had history of abortion (one abortion in 12 patients, 2 abortions in 2 patients and 3 abortions in 2 patients) except one patient. All of patients were on regular prophylaxis (10 IU every 2 weeks) for 24–62 months, every 4 weeks before pregnancy and every 2 weeks during pregnancy. The patients were followed for any bleeding episodes and also evaluated for bleeding score before and after prophylaxis according on standard form.

The monochloroacetic acid test was normal in all patients during prophylaxis.

The bleeding score was significantly decreased from 11–16 (mean 12) to 1–2 (mean1.5) starting prophylaxis. Two bleeding episodes were recorded in 2 patients, one subject in 11 patients and 3 patients didn’t experience any bleeding episodes. All of patients had successful delivery at 36 weeks gestation without any significant coagulation complications.

Pregnancy loss is a main complication in women with FXIII deficiency and prophylaxis is critical for pregnancy maintenance in these patients. In this study we experienced a successful pregnancy maintenance and delivery in Iranian women with FXIII deficiency. It is recommended to precise detection and diagnosis of FXIII in women with coagulation disorders for appropriate prophylaxis and prevention of pregnancy loss.

No relevant conflicts of interest to declare.