Burkitt lymphoma following renal transplantation

A 43-year-old woman presented with a mass in her right axilla and a left third cranial nerve palsy. She had a past medical history of familial juvenile hyperuricaemic syndrome and had received a cadaveric renal transplant 2½ years prior to presentation. Imaging of her brain was unremarkable, but computed tomography (CT) scan of her body showed bilateral axillary and cervical lymphadenopathy along with several hepatic lesions. The renal allograft also appeared abnormal, containing multiple low attenuating lesions consistent with lymphomatous involvement (top left). An axillary lymph node biopsy showed the classical ‘starry sky’ appearance of Burkitt lymphoma (top right). In addition, the tumour cells were positive for CD20, CD10, BCL6 and Epstein–Barr virus (EBV) encoded RNA (bottom left). These findings were consistent with the finding of 3·5 million copies/ml of EBV DNA by polymerase chain reaction (PCR) on the peripheral blood. Bone marrow examination showed involvement by the tumour (aspirate – inset). Cytogenetic analysis revealed t(8;22)(q24;q11), a variant of the t(8;14) (bottom right). She was diagnosed with a post-transplant lymphoproliferative disorder (PTLD) and was treated with reduction of immunosuppression in combination with alternating regimens of cyclophosphamide, vincristine, doxorubicin and methotrexate, along with etoposide, ifosfamide and cytarabine (CODOX-M/IVAC). EBV DNA was undetectable by PCR 6 weeks into treatment, together with resolution of the left third cranial nerve palsy. Serial CT scans showed regression of disease, and resolution of the changes within the renal allograft that continued to function well. Current World Health Organization criteria recognise three stages of PTLD; early lesions, polymorphic, and monomorphic PTLD. Monomorphic PTLDs are classified as lymphomas with the majority being diffuse large B-cell lymphomas. Burkitt lymphoma is less commonly encountered. Treatment options for PTLD include reduction of immunosuppression, rituximab monotherapy, and combination chemotherapy.