The Evolving Dilemma of Factor XI in Pregnancy: Suggestions for Management

Abstract

(Anesth Analg. 2018;126:2032–2037) Factor XI (FXI) deficiency or hemophilia C is an autosomal inherited multigenic bleeding disorder that can cause clinically significant hypocoagulability and bleeding, the severity of which does not correlate to the measured level of FXI. In this case study and discussion, the authors presented a 36-year-old pregnant patient of Ashkenazi Jewish descent with FXI deficiency, who had no history of excessive bleeding in multiple prior pregnancies. Before entering the authors’ practice, the patient had 4 previous uncomplicated vaginal deliveries, and denied any history of heavy menses or periods of heavy bleeding. In 2008 and 2012, the patient had 2 successful vaginal deliveries with no transfusions or noted postdelivery complications, with an estimated blood loss ranging from 250 to <300 mL. For her seventh pregnancy, a routine hematology evaluation advised that the patient “should be monitored. for excess bleeding peripartum.and given fresh frozen plasma (FFP) if needed.” Laboratory evaluation revealed an FXI level at 3% and an elevated activated partial thromboplastin time (aPTT) of 54.2 seconds. She prophylactically received 2 units of FFP, with a measured aPTT of 45 seconds after infusion, and the underwent a vaginal delivery of a healthy infant with an estimated blood loss of <250 mL. The patient was discharged from the hospital on postpartum day 1 with no complications.