ABSTRACT
Factor XI (FXI) deficiency is one of the rarest coagulation disorders with a frequency of 1:1,000,000. A 30-year-old woman, diagnosed with FXI deficiency at the age of 4, was admitted to our maternity service at 25 weeks of gestation. The patient had no history of hemorrhage or abnormal bleeding, and the clinical examination was unremarkable. Antenatal care was also normal. The parturient was admitted in early labor at 38 weeks of gestation. Despite the absence of clinical hemorrhagic syndrome, a transfusion of fresh frozen plasma combined with tranexamic acid was initiated once the active stage of labor was started. Management of FXI deficiency in pregnant women is a challenge due to its unpredictable bleeding tendency, and careful planning and knowledge of appropriate hemostatic management is pivotal for their care.
