Late-onset Epstein-Barr virus-negative post-transplant lymphoproliferative disorder presenting as ulcerated necrotic papules and nodules in a renal transplant patient.

ABSTRACT

We report a case of B-cell post-transplant lymphoproliferative disorder in a 57-year-old female 19 years postrenal transplant patient who presented with multiple, progressive, painful ulcerated necrotic papules and nodules over the left leg. Histopathological examination showed diffuse infiltration of the dermis by large atypical B-lymphoid cells, with a negative in situ hybridization test for Epstein-Barr virus. Gastrointestinal involvement was evident by the presence of atypical lymphoid cells in the peritoneal fluid. She only had partial response to localized radiotherapy and intravenous rituximab and died 4 months later of septicaemia. Unusual features highlighted in this case include the very late onset of disease 19 years post-transplant, Epstein-Barr virus negativity and the aggressive course of disease that did not respond to the reduction of immunosuppression, localized electron beam therapy and intravenous rituximab.