Background Post‐transplant lymphoproliferative disorder (PTLD) is a rare but serious complication of chronic immunosuppression in transplant recipients. Although previously thought to occur primarily in the first year post‐transplant, emerging data suggests a second peak in incidence in those who survive greater than 10 years after transplantation. Primary central nervous system (CNS) PTLD is an exceedingly rare subset of this disease, which can present with nonspecific neurologic symptoms, leading to diagnostic delays. We report a case of rapidly progressive very‐late‐onset primary CNS PTLD in a kidney transplant recipient. Case Presentation A 71‐year‐old woman with a history of renal transplantation 15 years prior, maintained on long‐term immunosuppression with prednisone and mycophenolate presented after a ground level fall. She endorsed progressive neurologic deficits, which had begun as mild right hand weakness. This rapidly progressed over the course of 3 weeks to encompass extreme lethargy, decreased oral intake, difficulty swallowing, right upper extremity immobility and right lower extremity weakness, and inability to bear weight. Imaging revealed multifocal enhancing brain lesions, with histopathology and immunohistochemistry confirming EBV‐positive monomorphic diffuse large B cell lymphoma, consistent with primary CNS PTLD. Despite initiation of high‐dose methotrexate, dexamethasone, and rituximab, the patient′s condition rapidly deteriorated, ultimately resulting in her passing within 2 months of initial neurologic deficit. Conclusion This unique case highlights the diagnostic challenges and aggressive course of a patient with very‐late‐onset primary CNS PTLD. Although PTLD was often thought to primarily occur soon after transplantation, emerging literature has established a growing number of late onset cases. Therefore, with increasing long‐term survival in transplant recipients, physicians should maintain a high index of suspicion for late onset CNS PTLD in individuals from this population who present with new neurologic symptoms, even decades after transplantation. Early imaging and brain biopsy remain essential for timely diagnosis and initiation of treatment.
Ghanem et al. (Thu,) studied this question.