Management of intrapartum bleeding risk in a case of Factor XI deficiency

Factor XI (FXI) deficiency is a congenital clotting disorder affecting 1 in 100,000 people worldwide. Severe deficiencies are defined as patients with FXI ≤ 20% of the normal, normal being defined between 60 -70 UI/dl. However levels are often not representative of level of activity. Due to this weak correlation between FXI levels and bleeding tendency, patients are often categorized into ‘bleeder’ phenotypes using bleeding symptoms such as heavy menstrual bleeding, bleeding after dental/surgical procedures, mucous membrane bleeding and easy bruising. However, as is often the case in medicine, FXI levels and historical care can often become the primary guide for treatment particularly in acute situations such as happened in this case.

This is compounded in pregnant populations were interpreting the stability of FXI levels is difficult as studies show either stable concentrations or small fluctuations making levels even more inaccurate for care planning.