Treatment outcome of post-transplant lymphoproliferative disorder in paediatric liver transplant recipients using rituximab-based regimen

Abstract

PURPOSE/OBJECTIVE: Post-tranplant lymphoproliferative disorder (PTLD) has a higher incidence in children than adults due to higher rate of seronegativity against Epstein-Barr virus. We reviewed the treatment results of PTLD in paediatric liver transplant recipients. MATERIALS AND METHODS: Clinical data of consecutive cases of PTLD diagnosed in paediatric liver transplant recipients in Queen Mary Hospital between 1-Jan-1999 and 31-Dec-2011 were reviewed up to the date of 31-Dec-2012. RESULTS: Twelve cases of PTLD (5 males; 7 females) were diagnosed during the study period. The majority had congenital biliary atresia and underwent liver transplantation before two years of age. The median age of diagnosis was 2.3 years (range, 0.8 to 9.5 years) and the median duration from time of transplantation to diagnosis was 10 months (range, 2 to 64 months). The median follow up duration was 3.7 years (range, 0.01 to 11.2 years). The clinical presentation varied from chronic infectious mononucleosis to frank lymphoma involving nodal or extranodal sites such as the liver graft and gastrointestinal tract. The histologic types comprised of monomorphic B cell (DLBCL, n ? 4; Burkitt, n ? 1), polymorphic B cell (n ? 3), T cell (n ? 1) and IM-like (n ? 3) PTLD. All had elevated plasma EBV loads at diagnosis. Treatment consisted of 4 weekly doses of rituximab at 375 mg/m2/dose for IM-like and polymorphous PTLD and low dose chemotherapy (up to 6 cycles of COP) in addition to 4 doses of rituximab for monomorphic PTLD. The 5-year event-free survival and overall survival were 58.3% and 66.7%, respectively. Three cases died of aggressive disease before treatment (2 monomorphic and 1 polymorphic PTLD presenting as leukaemia) and the case of T cell PTLD died after disease relapse. CONCLUSIONS: Vigilant monitoring for development of PTLD in paediatric liver transplant recipients is indicated. Rituximab-based regimen offers an effective treatment strategy.