2025 Volume 60 Issue 2 Pages 123-129
【Background】 Post-transplant lymphoproliferative disorder (PTLD) is a lymphoid or plasmacytic proliferation that occurs in association with immunosuppression and is recognized as a relatively rare but serious complication following solid organ transplantation.
【Case Presentation】 A woman in her 50 s developed type 1 diabetes in her teens and underwent living-donor kidney transplantation followed by deceased-donor pancreas transplantation. Due to recurrent type 1 diabetes in the transplanted pancreas, she received three islet transplantations. Five years after the last transplantation, she presented with persistent fever. PET-CT revealed lymphadenopathy in the mesentery and axilla, and biopsy confirmed a diagnosis of diffuse large B-cell lymphoma (DLBCL) -type PTLD.
【Treatment and Outcome】 Immunosuppressive therapy was adjusted without complete discontinuation. Four cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) therapy against PTLD resulted in partial remission, so subsequent four cycles of DA-EPOCH-R (dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, hydroxydaunorubicin, and rituximab) therapy led the PTLD to complete remission. Throughout the treatment, islet graft function was preserved. Therefore, no evidence of PTLD recurrence was observed during follow-up.
【Conclusion】 This is the first reported case of PTLD after islet transplantation in Japan. Careful adjustment of immunosuppressive therapy enabled disease remission while preserving islet graft function. This case provides important insights into the management of PTLD following islet transplantation.
