Severe acute allograft rejection 22 years after liver transplantation

Early acute cellular rejection occurs commonly within the first month after liver transplantation, is easily reversible with treatment, and has little impact on long-term graft survival. In contrast, late allograft rejection, typically defined as occurring after 3-6 months post-transplantation, can lead to the development of chronic rejection and graft loss. Alloreactivity and the risk of rejection decreases with time, since transplantation and many long-term liver transplant recipients can maintain graft function with minimal immunosuppression. We describe a case of acute allograft rejection 22 years and 5 months after liver transplantation. The patient, transplanted for biliary atresia as a young child, had three prior episodes of allograft rejection: two within the first month of transplantation, and one 4 years after. Subsequent to this, she had normal liver biochemistry and was maintained on minimal immunosuppression for many years. Following a diagnosis of immune thrombocytopenic purpura and marginal zone lymphoma, she was switched from tacrolimus to mycophenolate monotherapy, and subsequently developed significantly elevated liver enzymes, and biopsy confirmed severe acute allograft rejection. Our experience demonstrates that despite the liver being an immunotolerant organ, which often can be maintained with minimal immunosuppression post-transplantation, acute allograft rejection can occur at any stage and should always be considered as a possible cause of liver biochemistry abnormalities.