Case report for management of pregnancy and delivery in severe factor XII deficiency

Abstract

Factor XII deficiency is rare bleeding disorder and theoretically may carry either bleeding risk as a result of reduced coagulation factors or thrombosis risk as a consequence of reduced fibrinolytic activity. The incidence of factor XII deficiency is relatively low at 1 in 1,000,000 people. The aim of reported case is to emphasise the importance of multidisciplinary approach in the management of pregnancy and delivery in the women with severe factor XII deficiency. A woman with severe factor XII deficiency was successfully treated during her two pregnancies and deliveries. All coagulation tests were performed with standard Siemens reagents on the coagulometer Dade Behring BCS XP. A test for platelet aggregation for follow up of Aspirin treatment was performed on Siemens Innovance PFA-200 with standard Siemens collagen/epinephrin test cartridge. During her two pregnancies, the woman with severe factor XII deficiency was regularly followed up in the Center for haemophilia in the Institute for Transfusion Medicine. According to coagulation tests in the first pregnancy she was treated with low molecular weight heparin in the postpartum period, but in the second pregnancy it was necessary to be treated in the first and third trimester, as well as in the postpartum period. Bleeding prevention due to delivery was performed with tranexamic acid. Two pregnancies were successfully finished with vaginal delivery without any bleeding or thrombotic complications. Regular follow up during pregnancy is necessary for women with bleeding disorders in Comprehensive Haemophilia Treatment Centre. Multidisciplinary approach is crucial for successful bleeding disorders care