Aim: Primary central nervous system post-transplantation lymphoproliferative disorder (PCNS-PTLD) is a rare complication of immunosuppresion. Data is limited to case series, with median survival of 47 months reported in the literature. We aimed to identify our cases of PCNS-PTLD and describe clinical and pathological features and outcomes.
Methods: Search of Unit database and Australia and New Zealand Dialysis and Transplant Registry plus review of case records. We included recipients transplanted between 1990 and 2016 who were followed at our unit.
Results: Ten patients with PCNS-PTLD and no evidence of systemic PTLD were identified and examined. Median age was 52 (range 31-79). Median time to diagnosis after transplant was 13 years (range 1-36). Pathology from cerebral biopsy showed Epstein-Barr virus (EBV)-positive large B cell lymphoma in all patients. Follow-up was complete for all but one patient (lost to followup since 2011). Of these, four died: one was palliated after diagnosis of PCNS-PTLD due to multiple comorbidities. Another withdrew treatment three years after diagnosis aged 82 due to frailty. A third nursing-home patient died three years following PTLD from aspiration pneumonia. The forth patient withdrew dialysis after a prolonged admission from presumed progressive multifocal leukoencephalopathy 16 years post PTLD. Three patients remained in remission at time of death. All six surviving patients remain in remission and four retain graft function with a median eGFR of 54ml/min/1.73m2 (range 51-63). Two patients incurred graft loss, one at the time of PTLD diagnosis and the second at twelve months caused by chronic allograft nephropathy, however the second was re-transplanted 7 years after curative therapy. Treatment varied between patients and included surgical resection of single lesions, rituximab with systemic and/or intrathecal chemotherapy, localized or whole brain radiotherapy and reduction of immunosuppression.
Conclusions: Over half PCNS-PTLD cases remain in remission, most of whom have ongoing graft function. Younger patients with less co-morbidity had better survival. Our data suggests that patients can maintain long-term remission and survive without loss of their renal graft.  Long term follow-up of this cohort and others are needed to establish more accurate survival rates.