Acquired factor XI deficiency: a rare complication after liver transplantation

A majority of coagulation factors are synthesized in the liver. Factor XI (FXI) deficiency (Rosenthal syndrome) is one of the rare inherited coagulation disorders with an extremely low risk of transmission by liver transplantation (LT).

We report here the case of a 50-year-old man who unknowingly acquired FXI deficiency by LT. During 1 year of post-transplant follow-up, his activated partial thromboplastin time (aPTT) remained prolonged, but he did not develop bleeding complications. The patient required retransplantation due to chronic rejection and is currently doing well 4 years after his first liver transplantation.

The presence of a prolonged aPTT in a deceased donor should raise suspicion for the presence of rare coagulation factor deficiencies. During urgent, lifesaving procedures such as LT, it may be impossible to avoid transmission. Awareness of this possibility will allow early detection and management.