Bleeding and liver transplantation outcomes in haemophilia

Background Hepatitis C is the major cause of end‐stage liver disease and the major indication for orthotopic liver transplantation ( OLT x) in individuals with haemophilia. Aim To assess the epidemiology and outcomes of OLT x in U.S. haemophilia patients. Methods We investigated haemophilia liver transplant recipients between 1993 and 2012, using the Nationwide Inpatient Sample, identified by ICD 9 code 50.59. Results Of the 11 267 (weighted n = 54 691) patients undergoing OLT x, 44 (0.4%; weighted n = 213) had haemophilia. Those with haemophilia were more likely than non‐haemophilic OLT x recipients to have bleeding complications (45.3% vs. 31.5%, P = 0.009) and hypovolemic shock (7.0% vs. 1.1%, P < 0.0001). They also had a significantly higher incidence of HIV (24.8% vs. 0.5%, P < 0.005), hepatitis B (16.2% vs. 7.9%, P = 0.04) and vitamin K deficiency (2.1% vs. 0.02%, P < 0.001). In spite of these differences, there was no difference in in‐hospital mortality between haemophilic and non‐haemophilic recipients (6.8% vs. 6.2%, P = 0.9). In multivariate logistic regression, bleeding complications in haemophilia increased the risk of in‐hospital mortality by more than 3‐fold ( P < 0.0001), and disseminated intravascular coagulation increased the risk of bleeding complications in haemophilic recipients by over 10‐fold ( P < 0.0001). Conclusions Bleeding complications are common in haemophilia OLT x recipients. Thus, aggressive correction of coagulation defects in this group may be a medically sound approach to reduce complications and mortality associated with OLT x.