High incidence of recurrence and hematologic events following liver transplantation for Budd-Chiari syndrome

BACKGROUND: Most cases of Budd-Chiari syndrome (BCS) in Western countries are related to underlying hematologic diseases with inherent thrombogenic propensity. We evaluated the long-term outcome, risks for recurrent disease, and other hematologic complications following orthotopic liver transplantation (OLT) for BCS. METHODS: Clinical data from 11 consecutive patients with BCS who underwent OLT were retrospectively reviewed. Four patients had a prior transjugular intrahepatic portosystemic shunt and one had a surgical shunt procedure. All patients were started on intravenous heparin within the first 24 h following OLT. All except one patient who had protein C deficiency were maintained on long-term oral anticoagulation. RESULTS: The Kaplan-Meier survival rates at 1, 5 and 10 yr were 81, 65 and 65%, respectively. Three patients developed BCS recurrence, including two who died as a consequence of rapid graft failure within days after OLT. Three patients developed other thrombotic events, including splenic vein thrombosis associated with gastric variceal hemorrhage requiring splenectomy, portal vein thrombosis and pulmonary embolism. Four patients experienced severe bleeding complications within 7 d after OLT requiring exploratory laparotomy. One patient died after transformation of polycythemia vera to acute myelogenous leukemia at 2.1 yr after OLT. CONCLUSION: We observed a high incidence of recurrent BCS and complications related to the underlying hematologic disorder or anticoagulation after OLT for BCS. The present series also included the first two cases of rapid recurrence of BCS and graft failure within days after OLT.