Post‐transplant Lymphoproliferative Syndrome in the Pediatric Liver Transplant Population

Abstract

Post‐transplant lymphoproliferative disease remains a complication with a high morbidity and mortality. The present study examined 291 pediatric liver transplants performed in 263 children from October 1984 to December 1999. Post‐transplant lymphoproliferative disease has an overall incidence of 12%. Tacrolimus and cyclosporine had a similar incidence of post‐transplant lymphoproliferative disease. Fifty‐six per cent of patients who developed post‐transplant lymphoproliferative disease were Epstein–Barr virus negative at the time of transplantation. Mean time of conversion to Epstein–Barr virus positivity was 1.1 years after liver transplantation. Ten per cent of those who developed post‐transplant lymphoproliferative disease never had Epstein–Barr virus detected. Mean time from Epstein–Barr virus positivity to detection of post‐transplant lymphoproliferative disease was 2.68 years, and 3.13 years from liver transplantation (OLTx) to post‐transplant lymphoproliferative disease. There was a 35% incidence of mortality. Deaths occurred a mean of 0.76 years after diagnosis of post‐transplant lymphoproliferative disease. Most cases of post‐transplant lymphoproliferative disease had extranodal location. There was one recurrence in 10% of patients, and two in 3%. All recurrent cases were seen in recipients who became Epstein–Barr virus positive after transplantation. There has been a decrease in the incidence of post‐transplant lymphoproliferative disease from 15% to 9% to 4%. Post‐transplant lymphoproliferative disease should be diagnosed promptly and treated aggressively. The best treatment, however, seems to be prevention, starting in the immediate postoperative period. Survivors should be monitored for both recurrence of post‐transplant lymphoproliferative disease and acute cellular rejection. Post‐transplant lymphoproliferative disease remains a complication with a high morbidity and mortality. The present study examined 291 pediatric liver transplants performed in 263 children from October 1984 to December 1999. Post‐transplant lymphoproliferative disease has an overall incidence of 12%. Tacrolimus and cyclosporine had a similar incidence of post‐transplant lymphoproliferative disease. Fifty‐six per cent of patients who developed post‐transplant lymphoproliferative disease were Epstein–Barr virus negative at the time of transplantation. Mean time of conversion to Epstein–Barr virus positivity was 1.1 years after liver transplantation. Ten per cent of those who developed post‐transplant lymphoproliferative disease never had Epstein–Barr virus detected. Mean time from Epstein–Barr virus positivity to detection of post‐transplant lymphoproliferative disease was 2.68 years, and 3.13 years from liver transplantation (OLTx) to post‐transplant lymphoproliferative disease. There was a 35% incidence of mortality. Deaths occurred a mean of 0.76 years after diagnosis of post‐transplant lymphoproliferative disease. Most cases of post‐transplant lymphoproliferative disease had extranodal location. There was one recurrence in 10% of patients, and two in 3%. All recurrent cases were seen in recipients who became Epstein–Barr virus positive after transplantation. There has been a decrease in the incidence of post‐transplant lymphoproliferative disease from 15% to 9% to 4%. Post‐transplant lymphoproliferative disease should be diagnosed promptly and treated aggressively. The best treatment, however, seems to be prevention, starting in the immediate postoperative period. Survivors should be monitored for both recurrence of post‐transplant lymphoproliferative disease and acute cellular rejection.