The obstetric experience of women with factor XI deficiency

Objectives. To assess the obstetric outcome in women with factor XI (FXI) deficiency. Design. Retrospective review of medical records. Setting. Tertiary referral university hospital. Population. Women with FXI deficiency. Method. Review of pregnancies over a 10-year period (1997-2006). Main outcome measures. Pregnancy outcome, mode of delivery, changes in FXI levels during pregnancy, use of prophylaxis during labor and delivery, antepartum hemorrhage, and postpartum hemorrhage (PPH). Results. There were 61 pregnancies among 30 women with FXI deficiency (two severe, FXI level 15-20 IU/dL, and 28 partial, FXI level 20-70 IU/dL) resulting in 49 live births (two sets of twins), eight miscarriages, and six terminations of pregnancy. The modes of delivery included 38 spontaneous vaginal deliveries, three instrumental deliveries, and six cesarean sections (two emergency and four elective). No significant change in FXI levels was observed during pregnancy. Intrapartum prophylaxis with FXI concentrate or tranexamic acid was given in 19 deliveries where the mother had a positive bleeding history. Four women had excessive bleeding related to pregnancy loss and three experienced antepartum bleeding. All these women had a positive bleeding history. There were five (11%) primary and five (11%) secondary PPHs among seven women including four with a positive bleeding history. Conclusion. Women with FXI deficiency, particularly those with a positive bleeding history, are at risk of bleeding complications related to miscarriage or childbirth. The unpredictable nature of their bleeding tendency demands careful planning and close collaborations between obstetricians and hematologists.