Abstract
INTRODUCTION: Hyperacute rejection leading to hepatic necrosis or intrahepatic bile duct stricture in ABO incompatible living-donor liver transplant (ABO-i LDLT) has been reported many times. With the advent of rituximab, the incidence of these complications has decreased significantly. However, consecutive biliary disruption after ABO-i LDLT has rarely been reported. PRESENTATION OF CASE: A female in her 50s with blood type A was admitted to our hospital for ABO-i LDLT due to failure of a graft (refractory ascites [Child-Pugh C(10), MELD 9]) that had been primarily transplanted 20 years ago from her ABO-identical father. Since the living donor was her husband with blood type B, rituximab was administered for ABO-i re-LDLT. After the LDLT, the patient recovered quickly despite bile leakage at the biliary anastomosis. Subsequently, the bile duct of the graft liver was serially disrupted with a bile lake, which required multiple instances of biliary drainage. A liver biopsy was performed and did not show any C4d staining on 195 post-transplant days. The patient ultimately developed sepsis due to cholangitis and expired at 11 months after the re-LDLT and finally C4d was positive on post-mortem biopsy. DISCUSSION: Advances in ABO-i LDLT, particularly with rituximab, have reduced complications, but consecutive bile duct disruption remains challenging. Despite positive donor-specific antibody, early rejection markers were absent, suggesting complex mechanisms of complication. CONCLUSION: We herein report a rare case as an important observation that may aid in preventing and treating potentially fatal complications after ABO-i LDLT.