A Case of Post-transplant Lymphoproliferative Disorder Developed 45 Years after Kidney Transplantation

Introduction: Post-transplant lymphoproliferative disorder (PTLD) affects 1-3% of renal transplant recipients, with the majority of cases occurring within the first year or around 5 years post-transplant. PTLD presents with a variety of symptoms depending on the affected organs and requires treatment including reduction or discontinuation of immunosuppressive agents. Very late-onset PTLD (VL-PTLD), developing more than 10 years after transplantation, is rare and requires different management strategies from early or late-onset PTLD due to its characteristics. Case Description: A 61-year-old male pharmacist, who had undergone kidney transplant 45 years ago due to purpura nephritis and was maintained on AZA+PSL, developed fever and shortness of breath. Blood test showed inflammation and worsening kidney function but EBV was negative. Computed tomography showed multiple nodules and masses in lung and liver, and bronchoscopy revealed a tumor exposed in the left bronchus. A lung biopsy confirmed monomorphic PTLD (diffuse large B-cell lymphoma type, stage IVB). Treatment was initiated with change in immunosuppressive agents from AZA to everolimus and chemotherapy (R-CHOP). His symptoms improved and he was discharged on day 45. However, sudden bleeding from his airway led to cardiopulmonary arrest and death on day 66. Discussion: Three percent of post-kidney transplant patients at our hospital have presented with PTLD, of which this case was the only case of VL-PTLD with the oldest age. It has been reported that VL-PTLD cases are often older and on classical immunosuppressive agents such as AZA, and present monomorphic type pathologically. Previous reports have indicated that the 1- and 5-year survival rates for VL-PTLD are 56% and 29%, respectively, and poor prognostic factors include age 65 or older, male gender, and CyA+AZA maintenance therapy. Continued attention to PTLD should be paid even long after kidney transplantation. Further case collection and research is needed to establish the optimal treatment strategy for VL-PTLD.