Post-Transplant Lymphoproliferative Diseases: A Case Report And Discussion Of Management

Post-transplant lymphoproliferative disorders (PTLD) are lymphoid or plasmacytic proliferations that develop in immunosuppressed individuals, particularly in recipients of solid organs, bone marrow, or stem cell allograft. Although rare in the general population, the prevalence of PTLD is increasing among transplant patients, particularly as transplantations have become more frequent procedures in recent years. PTLD are very closely associated with Epstein-Barr virus infection, with up to 80% of all PTLD cases testing positive for Epstein-Barr virus. Histologically, PTLD have three main subtypes as classified by the World Health Organization: early lesions, polymorphic PTLD and monomorphic PTLD, which can be regarded as lying on a pathological continuum. While there are currently no data from phase III clinical trials to guide optimal treatment of PTLD, evidence from phase I and II studies, retrospective analyses and case series, as well as expert opinion, indicate the effectiveness of immediate reduction in immunosuppression as first-line therapy, particularly in early-stage disease. When reduction in immunosuppression is inadequate, the evidence shows that addition of rituximab and / or chemotherapy is a useful and effective option. Radiation has limited indication in PTLD except in localised disease and for palliation. This article describes a case of PTLD in a female patient with end-stage renal failure due to lupus nephritis, who had previously received a cadaveric renal transplant. Her management is discussed with some reference to the published literature and current opinion.