How I Treat How I treat posttransplant lymphoproliferative disorders

A 19-year-old woman with end-stage renal failure due to congenital kidney disease underwent a living-donor renal transplant in 2006. Eight years later, she was diagnosed with EBV MYC translocation– confirmed BL-PTLD stage IVB (Figure 1) while on treatment with mycophenolate mofetil (MMF), tacrolimus, and low-dose steroids. EBV viral load was very high (5.84 log IU/mL). Due to the aggressive presentation, immune suppression was completely discontinued and immunochemotherapy with rituximab and CHOP (cyclophosphamidedoxorubicin-vincristine-prednisone) was started. Partial metabolic response was obtained after 4 cycles of chemotherapy (Figure 1). However, at the start of the sixth cycle, the patient complained of headache; clinical examination showed a peripheral facial nerve paresis. Morphologic examination of the cerebrospinal fluid confirmed the presence of a BL relapse with high EBV viral load, whereas brain magnetic resonance imagingdid not reveal cerebral or cerebellar involvement.After treatment with intrathecal methotrexate, clinical symptoms disappeared, with normalization of the cerebrospinalfluid. Consolidation therapywith 4 cycles of high-dose methotrexate (3.5 g/m) was given. A final evaluation with F-FDG–PET/CT, bone marrow examination, and brain magnetic resonance imaging showed complete remission. Lumbar puncture, however, showed the presence of meningeal disease, and rescue treatment with intrathecal rituximab and systemic etoposide and cytarabine was initiated, leading to an ongoing complete remission.