A CASE REPORT WITH SEVERE CONGENITAL FACTOR XIII DEFICIENCY AND AN UNCOMPLICATED PREGNANCY AND BIRTH PROCESS

Abstract

Introduction: Factor XIII deficiency is an extremely rare type among bleeding diathesis. In factor XIII deficiency, normal results of coagulation screening tests are expected. It usually does not cause spontaneous bleeding. Apart from bleeding diathesis, it may cause delayed wound healing and recurrent spontaneous abortions in women. Here, we present a 32-year-old case with severe congenital factor XIII deficiency who had an uncomplicated pregnancy and birth with regular replacement therapies. Case report: A 32-year-old patient with severe congenital factor XIII deficiency, who had a history of spontaneous abortion at the 11th week of her first pregnancy, applied to our center with a request for childbirth. It was learned that the factor XIII levels of the patient could not be measured, that she was using plasma-derived FXIII concentrate at a dose of 25 units/kg every time once a month, and in cases where this could not be obtained, 5 units/kg cryoprecipitate was given instead. After the completion of the pre-pregnancy assessments, starting 3 months before the planned pregnancy and continuing for the whole pregnancy and for 3 months after birth, 25 units/kg plasma-derived concentrate at a dose of 25 units/kg was applied each time and every two weeks, and in cases where this could not be provided, the follow-up was continued by applying cryoprecipitate at a dose of 5 units / kg instead. During this whole process, FXIII levels ranged between 70% and 100%. The patient, who developed an abortion risk due to decidual bleeding in the first trimester, was hospitalized and an additional 25 units / kg plasma-derived FXIII concentrate was administered and a parenteral dose of 30 mg / kg tranexamic acid was applied until the signs of decidual bleeding disappeared. An additional 50 units/kg dose of plasma-derived FXIII concentrate was administered to the patient 30 minutes before birth who had a planned delivery by cesarean section at 38 weeks of gestation, and 30 mg/kg parenteral tranexamic acid was administered for 7 days following the delivery. FXIII level was detected 50% in the child of a healthy, 3500-g born boy. The patient and her baby, who are in the first year after birth, are followed up without any complications, and prophylactic plasma-derived FXIII concentrate or cryoprecipitate is administered to the patient once a month. Discussion and Conclusion: Inherited bleeding diathesis lead to an increased risk of bleeding and abortion in obstetric patients. Factor XIII deficiency is an extremely rare type among them. FXIII has a role in angiogenesis as well as hemostasis. Therefore, wound healing and tissue repair are impaired in Factor XIII deficiency. The risk of premature separation of the placenta, miscarriage especially in the first trimester, and postpartum uterine bleeding are increased in FXIII deficiency. Tranexamic acid can be used safely in obstetric patients with bleeding diathesis. It may be possible to ensure that patients with factor XIII deficiency have an uncomplicated pregnancy and delivery with regular follow-ups, regular prophylactic factor preparations, plasma replacements if they are not found, and in cases of bleeding, with additional doses of factor preparations or plasma replacement applications with tranexamic acid.