A 15-year-old Japanese female with end-stage kidney disease, kidney cysts, and angiomyolipoma due to tuberous sclerosis complex (TSC) received an ABO-matched preemptive kidney transplantation from her father. Basiliximab induction therapy was done on days 0 and 4, and tacrolimus, mycophenolate mofetil, and methylprednisolone were administered. Six months later, cervical lymphadenopathy developed, and computed tomography revealed an abdominal mass. Epstein-Barr virus-positive T cell post-transplant lymphoproliferative disorders (PTLD) was diagnosed. The pathology showed a monomorphic type lymphoma, and the Ki-67 index, a cell proliferation marker, was above 90%. The patient received four courses of CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone) therapy, and tacrolimus was switched to everolimus, an inhibitor of the mammalian target of rapamycin (mTOR) pathway. Everolimus acts not only as an immunosuppressant, but also has an anti-tumor effect which may inhibit lymphoma development and proliferation. Three years later, the patient has shown no sign of PTLD recurrence. Her kidney function remains good, and a pathological examination detected no sign of rejection. In addition, her facial angiofibroma has improved. Although this study is based only on a single case observed over a short period of time, we consider everolimus to be a possible option in the treatment of PTLD after CHOP chemotherapy, especially in patients with TSC.
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