SUCCESSFUL MANAGEMENT OF SEVERE CONGENITAL FACTOR X DEFICIENCY DURING PREGNANCY AND LABOR WITH PCC IN TWO SISTERS

Abstract

Introduction: Factor X (FX) deficiency is an autosomal recessive disorder caused by quantitative or qualitative defects in the FX protein. FX deficiency has an estimated worldwide prevalence of one in 1000000. (1) Pregnancy in women with congenital FX deficiency has been associated with adverse fetal outcomes (abortion and preterm labor) (2,11). We report two cases of successful pregnancy with factor X deficiency. Case 1: A 29-year-old woman with congenital factor X deficiency and prior abortion on prophylaxis PCC every 4 weeks. She was treated with PCC 25unit/kg twice weekly during the pregnancy course. At week 32 of pregnancy, she presented with labor pain. Lab showed PT 20.7 PTT 52.5 INR1.5 Fibrinogen 3.8 Hb13.8 platelet 195 WBCs 7.6 factor X 0.15. She was given PCC 25 units/kg until a level of 0.4 was achieved. She delivered a healthy, 1.9 kg baby by normal vaginal delivery. The estimated blood loss was 150 ml. She then received FX 15 units/kg for 3 days postpartum to maintain FX level >30% and INR 30% and INR 04 iu/ml. They also recommend, to consider further PCC 10–20 iu/kg once daily to maintain FX activity >03 iu/ml for at least 3 days post-partum. (10) Conclusion: Prophylactic PCC resulted in excellent hemostasis in two of our patients, including one that delivered by C-section.