Post-transplantation lymphoproliferative disease in pediatric liver recipients in Taiwan.

BACKGROUND: Post-transplantation lymphoproliferative disorder (PTLD) is a heterogeneous, potentially life-threatening complication after liver transplantation in children. In this study, the disease characteristics, outcomes, and prognostic factors of PTLD were investigated.

METHODS: A retrospective, observational study was conducted on 16 pediatric liver transplant (LT) recipients who developed PTLD between February 2001 and December 2013 at a tertiary referral center in Taiwan. The disease characteristics and treatment outcomes of the patients were analyzed.

RESULTS: The median time from liver transplantation to the development of PTLD was 8 months. Early onset (<1 year post-transplantation) PTLD constituted 62.5% of the cases. PTLD location was frequently extranodal (81.3%) and mostly involved the gastrointestinal tract (68.8%). Histopathologic findings showed polymorphic PTLD in six cases (37.5%), diffuse large B-cell lymphoma in five cases (31.3%), and Burkitt's PTLD in two cases (12.5%). Early lesions in PTLD, T-cell lymphoma, and Hodgkin's lymphoma were observed in one case each (6.3%). Overall survival at 1- and 5-years post-PTLD diagnosis was 87.5% and 79.5%, respectively. St Jude's classification stage IV disease was associated with poor survival (hazard ratio [HR] = 13.37, P = 0.037). Two patients (12.5%) had chronic rejection after the treatment for PTLD and one patient (6.3%) developed graft failure.

CONCLUSIONS: PTLD is a major complication in pediatric LT recipients, but long-term survival is possible in most cases with an adequate treatment strategy. Stage IV disease is a major risk factor for poor survival in pediatric PTLD patients. During the management of PTLD, the possibility of chronic rejection and even graft failure should be considered.