Posttransplant lymphoproliferative disorder in pediatric liver transplantation.

The success rate of pediatric liver transplantation has improved in recent years. Advances in immunosuppression have reduced the risk of rejection, but have enhanced the risk of posttransplant lymphoproliferative disorder (PTLD). Since 1994, we have performed 197 orthotopic liver transplantations in 157 recipients younger than 15 years. Herein we have performed a retrospective study to review the incidence and clinical characteristics, along with the treatment and outcomes of PTLD diagnosed over this 14-year experience. We documented 8 cases of PTLD (5%), half of which occurred during the first 2 years posttransplantation; 5 presented with abdominal involvement and 2 with thoracic masses. The histological findings showed lymphoma in 6 cases. All were treated with reduction of immunosuppression and 2 received Rituximab. Three patients died, a mortality rate of 37.5%. One subject experienced rejection, and the others responded to treatment. PTLD is a life-threatening condition that requires a high index of suspicion, appropriate imaging, biopsy diagnosis, and prompt treatment to achieve positive results. Quantitative monitoring of Epstein-Barr virus load may be useful to detect a high-risk population.