Central Nervous System Post-Transplant Lymphoproliferative Disorder After Kidney Transplantation in an Epstein-Barr Virus-Seronegative Recipient Despite Serial EBV-DNA Monitoring: A Case Report.

BACKGROUND

Post-transplant lymphoproliferative disorder (PTLD) is a serious complication of organ transplantation that is strongly associated with Epstein-Barr virus (EBV) infection. Serial monitoring of peripheral blood EBV -DNA is widely used for early detection of PTLD; however, its sensitivity is limited, particularly in cases involving the primary central nervous system.

CASE PRESENTATION: A 23-year-old female patient underwent living-donor kidney transplantation from her mother and was classified as having standard immunological risk. The EBV serostatus was donor-positive/recipient-negative, and serial peripheral blood EBV-DNA monitoring was performed after transplantation. Despite the EBV-DNA levels remaining below 4 log10 IU/mL, the patient developed progressive neurological symptoms. Subsequent neuroimaging and histopathological evaluations led to the diagnosis of central nervous system PTLD (CNS-PTLD). Treatment consisted of immunosuppression reduction, localized radiotherapy, and systemic rituximab-based chemotherapy and resulted in complete metabolic remission.

CONCLUSION

Although EBV-DNA levels over 5 log10 IU/mL indicate an urgent concern for PTLD, this case highlights that CNS-PTLD can develop in high-risk transplant recipients even in the absence of significant EBV-DNAemia. Careful clinical assessment and prompt diagnostic evaluation of neurological symptoms are crucial for early diagnosis and appropriate management, particularly in donor-positive/recipient-negative kidney transplant recipients.