A Novel Framework for Factor Replacement in Patients Undergoing Liver Transplantation for End Stage Liver Disease

Background The management of coagulopathy and rebalanced hemostasis in end-stage cirrhosis is challenging. Additionally, a certain subset of patients presenting for transplant may have additional acquired or inherited coagulation disorders in addition to liver disease. In patients with these coagulation disorders, there is no updated guidance for isolated factor replacement. Inspired by a case at our institution, we conducted a pilot study to propose a framework to develop a goal-directed dosing strategy for factor repletion in patients presenting for liver transplantation. Methods To determine the level of coagulation factors and the between and within patient variation, factors FVII, FIX, FX, Antithrombin (AT), Protein C (PC), and Protein S (PS) were measured in 41 patients with end stage liver disease and undergoing liver transplantation. Results Coagulation factor data was collected from forty-one patients presenting for liver transplantation. The median level of all measured coagulation factors was below 80% (range 26–76%). The median levels varied widely in studied population (2.1-fold median interquartile range). Notably, measured levels of FIX were strongly correlated with other factors in individual patients: FIX versus FVII (R = 0.81, p < 0.001), FX (R = 0.74, p < 0.001), AT (R = 0.77, p < 0.001), and Protein C (R = 0.86, p < 0.001). Discussion Considering that the median levels were below 80%, for all factors, that levels varied widely among patients, and that levels were highly intercorrelated in an individual patient, it seems appropriate to propose and alternative and empiric target when substituting FIX in patients with hemophilia B before undergoing liver transplantation. We propose the average level of a set of highly inter-correlated coagulation factors as an empiric target when determining goal level for factor repletion.