Non-Hodgkin lymphoma (NHL) that develops following pediatric kidney transplantation is characterized as a post-transplant lymphoproliferative disorder (PTLD) and occurs at an incidence rate of 2-3%. The majority of cases among pediatric kidney transplant recipients are related to primary infection with Epstein-Barr virus (EBV), related to the ability to transform and immortalize B cells and widely proliferate due to the lack of relevant control of cytotoxic T cells in patients receiving immunosuppression following transplant.
Ryzard Grenda, MD, and colleagues reviewed NHL following pediatric kidney transplantation in Pediatric Nephrology [2022;37(8):1759-1773].
NHL may develop as a systemic disease or as a localized lesion. The clinical pattern is variable, ranging from nonsymptomatic disease to fulminating disease. Risk factors include young age of the transplant recipient, seronegative EBV status at the time of transplantation, and EBV mismatch between donor and recipient (D+/R-). The development of both early and late NHLs is impacted by immunosuppression.
Patients at risk require specific surveillance protocols that include monitoring of EBV viral load. However, according to the researchers, therapeutic decisions also require detailed histopathology diagnosis and evaluation of malignancy staging. The primary management tool is minimizing immunosuppression, followed by the use of rituximab in B-cell NHLs. Advanced NHLs require specific chemotherapeutic protocols, adjusted to lymphoma classification and staging.
Use of radiotherapy and/or surgical removal of malignant lesions is limited to the most severe cases. The outcome is variable and depends on risk factors and the timing of the diagnosis. In terms of graft function and patient survival, the outcome is generally positive in pediatric patients.
In survivors who lost the primary graft due to chronic rejection, retransplantation is possible, if performed a minimum of 2 to 3 years following graft loss. Candidates for retransplantation should undergo verification of malignancy-free status and immunity against EBV.