Introduction: Although the literature reports a low incidence of Burkitt Lymphoma (BL) as a post-transplant lymphoproliferative disorder (PTLD), this entity appears to be different from other monomorphic PTLDs (M-PTLDs), both in its aggressive clinical presentation and its distinct pathologic profile.

Case Report: Our case was a eleven-year old boy who was referred to the Transplant Department at Başkent University of Ankara at the age of 2 years, with “progressive familial intrahepatic cholestasis byler’s disease” presented with jaundice to develope since he was born. In our institution, he received liver transplant at the age of 26 months from his paternal uncle, and started to use tacrolimus based immunosuppressive regimen. The patient was EBV seronegative before liver transplantation. During his routine controls EBV-PCR got positive a month after his transplantation. Two months after liver transplantation he was treated with antiviral therapy (ganciclovir) for positive CMV antigenemia. Two acute rejection episodes, were treated with steroids. Seventeen months after liver transplantation, the computed tomography showed multiple portal masses and diagnosed as Burkitt lymphoma with liver allograft needle-guided biopsy. Bone marrow biopsy was also positive for tumor involvement. The tumor displayed the typical histological features of Burkitt’s Lymphoma and was markedly positive with insitu hybridisation for EBV. After the diagnosis of PTLD, the chemotherapeutic regimen consisted of etoposide 30mg, adriamycin 6,5mg, cyclophosphamide 60mg and prednisolone 4x7,5 mg has given. He is in complete remission, approximately 76 months after completion of the therapy.

Conclusions: There are only isolated case reports of Burkitt’s lymphoma presenting as PTLD in the literature. Because of the aggressive progression and the high turn over of tumor proliferation, it is important to start chemotherapy immadiately after the diagnosis of Burkitt lymphoma for the best outcome. Therefore it is very important and life-saving to diagnose Burkitt lymphoma as quickly as possible. Thus because of the close relationship between EBV and PTLD, we recommend a viral monitoring of EBV during follow-up of especially in pediatric LTx patients to prevent dramatic outcomes because of the possible development of PTLD.