Epidemiology and outcome of post-transplant lymphoproliferative disorders in pediatric liver transplant receipient

Abstract

BACKGROUND/OBJECTIVES: Post-transplant lymphoproliferative disorder (PTLD) has a higher incidence in children than adults due to high rate of Epstein-Barr virus seronegativity. We reviewed the epidemiology and outcome of PTLD diagnosed in pediatric liver transplant recipients. DESIGN/METHODS: To determine the cumulative incidence and review the clinical data of PTLD diagnosed in the cohort of pediatric patients (<18 years) who had liver transplantation performed between 1 January 2001 and 31 December 2012 in the major liver transplantation centre (Queen Mary Hospital) of Hong Kong. RESULTS: Among 75 cases of pediatric liver transplantation performed during the study period, 17 cases of PTLD (seven males) were diagnosed, giving cumulative incidence of 6.8% at 1 year and 22% at 5 years. Median ages of liver transplantation and diagnosis of PTLD were 9.5 months (range: 6–51 months) and 31.2 months (range: 10–114 months), respectively. Median duration from time of transplantation to diagnosis of PTLD was 18.4 months (range: 4.1–65 months). The series consisted of 10 infectious mononucleosis-like cases, 2 polymorphic, 4 monomorphic (3 Burkitt, 1 DLBCL) and 1 T cell PTLDs. One fulminant case (polymorphic PTLD, leukemic presentation) died prior to any treatment. The rest had reduction/cessation of immunosuppressive drugs. Three cases went into CR. Nine cases received rituximab only; eight went into CR. One case required a second course of rituximab for relapsed disease. Five cases received chemotherapy _ rituximab. Two cases entered CR and one case required further chemotherapy for relapsed disease. The case of T cell PTLD was salvaged by allogeneic stem cell transplantation. CONCLUSION: This series demonstrated the high incidence of PTLD in the young liver transplant recipients, supporting the need of regular clinical and viral surveillance for early detection. Most cases respond to reduction of immunosuppressive drug or specific treatment.