Post cardiac transplantation lymphoproliferative disorder presenting as t(8;14) Burkitt leukaemia/lymphoma treated with low intensity chemotherapy and rituximab

Background. Post-transplant lymphoproliferative disorder (PTLD) occasionally presents as Burkitt lymphoma/L3 leukaemia (BLL). Procedure. We reviewed records of cases of PTLD post-cardiac transplantation (1990-2007) occurring in our unit. Results. There were 15 episodes in 13 patients including four cases of EBV-driven Burkitt-type disease with t(8;14) translocations presenting with advanced stage disease. The first case was treated with a variety of low dose chemotherapy combinations. Despite problems during therapy he obtained complete remission, but died from complications of pre-existing cardiac allograft vasculopathy 7 months later. The subsequent three cases were treated with a UKCCSG low stage lymphoma protocol, NHL 9001 and Rituximab. They remain in complete remission. Conclusions. In the context of PTLD the prognostic significance of advanced stage EBV-driven BLL with the t(8;14) translocation may be different to that in immunocompetent children.