Successful management of severe congenital factor x deficiency during pregnancy and labor with pcc in two sisters

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 <1.5. No episodes of abnormal bleeding were observed during pregnancy, labor or post-partum. Case 2: A 36-years-old woman with congenital factor X deficiency and two prior abortions, on prophylaxis PCC every 4 weeks. She received prophylaxis PCC 25units/kg twice weekly during the course of this pregnancy. At week 38 of pregnancy, she delivered a healthy 3.2 kg baby by cesarean section (CS) after failing labor induction. Lab showed PT 23.7 PTT 50.4 INR1.7 Fibrinogen 2.3 CBC was normal.FX 0.13. She was given PCC 25 units/kg until a level of 0.4 was achieved. The estimated blood loss was 500 ml. She then received FX 15 units/kg for 7 days postpartum to maintain FX level >30% and INR <1.5. She was discharged on tranexamixc acid. No episodes of abnormal bleeding were observed during pregnancy CS or post-partum. Discussion: Although FX activity increases during normal pregnancy, levels usually remain insufficient for hemostasis at delivery in women with severe FXD (4,5,6). FX replacement therapy with PCC or FX concentrate may be required to treat or prevent bleeding in FXD.Therefore, a therapeutic dose of PCC 20–30 iu/kg is expected to increase plasma FX activity by 0.4–0.6 iu/ml. Further infusions at 1- to 2-d intervals may be required if sustained treatment is necessary (3). There are reports of FX replacement with PCC during pregnancy in women with previous adverse pregnancy outcomes (7,8) and FX replacement during labor with PCC or FFP, but with different regimens (9). Our patients were treated with PCC prophylaxis during pregnancy and 25 units/kg during labor. No bleeding nor thrombosis was seen in both cases. The British guidelines recommend PCC 20–40 iu/kg during the third trimester for women with history of bleeding and with FX activity <03 iu/ml with the goal of achieving FX activity >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.