Organ Specific Issues of PTLD – Liver

The incidence of post-transplant lymphoproliferative disorder (PTLD) following pediatric liver transplantation is decreasing, but it is still associated with significant morbidity and mortality. In children, PTLD mostly occurs early after transplant and is associated with Epstein-Barr virus (EBV). The risk factors for PTLD in children are young age, EBV seronegative status at the time of transplantation, and intense immunosuppression. The initial treatment consists of reduction of immunosuppression (RIS). Discontinuation of immunosuppression may be necessary in severe cases of PTLD. Some children stay off immunosuppression for years. Patients who experience rejection do respond well to corticosteroids, and chronic rejection is rarely seen. Mortality has been reported as high as 60%, although it seems to be dropping in recent reports. Patients who do not respond to RIS or discontinuation of immunosuppression usually have monoclonal monomorphic PTLD and require a combination treatment with anti-CD20 antibodies and chemotherapy.