Burkitt’s Lymphoma Presenting as Late-Onset Posttransplant Lymphoproliferative Disorder following Kidney and Pancreas Transplantation: Case Report and Review of the Literature

Abstract

Posttransplant lymphoproliferative disorders (PTLD) are a rare, but serious complication following transplantation. Late-onset PTLD are often associated with more monoclonal lesions and consequently have a worse prognosis. There are only isolated case reports of Burkitt’s lymphoma presenting as PTLD. We present an extremely rare, aggressive Burkitt’s lymphoma years after kidney and pancreas transplantation which was successfully treated with combination chemotherapy along with withdrawal of immunosuppression. The patient remains in complete remission more than 2 years after his diagnosis. We also provide a succinct review of treatment of various PTLD and discuss the role of Epstein-Barr virus infection in the pathogenesis of PTLD.

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“…The interval between liver transplant and development of posttransplant lymphoproliferative disorder (Burkitt lymphoma) in the present patient was consistent with other published pediatric cases (range, 6 to 107 mo after transplant). 4,[6][7][8] In 70% Burkitt lymphomas that occur after organ transplant, genes or gene products related to EpsteinBarr virus may be demonstrated within the tumor cells. 4 Burkitt lymphomas in transplant recipients may include tumors that stain positive or negative for Epstein-Barr virus, and the different types of Burkitt lymphoma may have different causes.…”

“…4,[6][7][8] In 70% Burkitt lymphomas that occur after organ transplant, genes or gene products related to EpsteinBarr virus may be demonstrated within the tumor cells. 4 Burkitt lymphomas in transplant recipients may include tumors that stain positive or negative for Epstein-Barr virus, and the different types of Burkitt lymphoma may have different causes. 5 The presence or absence of Epstein-Barr virus with the tumor is important for prognosis; Epstein-Barr viruspositive Burkitt lymphoma after transplant, which developed in the present patient, may respond well to reduction or cessation of immunosuppressive therapy or may have permanent complete remission without chemotherapy, but Epstein-Barr virusnegative Burkitt lymphoma has a very poor prognosis and may not respond to aggressive chemotherapy protocols.…”

“…5 The presence or absence of Epstein-Barr virus with the tumor is important for prognosis; Epstein-Barr viruspositive Burkitt lymphoma after transplant, which developed in the present patient, may respond well to reduction or cessation of immunosuppressive therapy or may have permanent complete remission without chemotherapy, but Epstein-Barr virusnegative Burkitt lymphoma has a very poor prognosis and may not respond to aggressive chemotherapy protocols. 4 There is a positive correlation between EpsteinBarr virus DNA level in the bloodstream after transplant and the development of posttransplant lymphoproliferative disorder, which suggests that monitoring viral load may be important after transplant. 4,[6][7][8][9][10] Subtyping also is important in patients diagnosed with posttransplant lymphoproliferative disorder.…”

“…4 There is a positive correlation between EpsteinBarr virus DNA level in the bloodstream after transplant and the development of posttransplant lymphoproliferative disorder, which suggests that monitoring viral load may be important after transplant. 4,[6][7][8][9][10] Subtyping also is important in patients diagnosed with posttransplant lymphoproliferative disorder. Burkitt lymphoma may have a high tumor growth rate and aggressive behavior, and early diagnosis is very important for effective treatment.…”

“…[1][2][3] The risk of developing non-Hodgkin lymphoma is increased 6-fold after transplant. 3,4 Burkitt lymphoma is an aggressive B-cell nonHodgkin lymphoma that is endemic in parts of Africa and New Guinea and occurs sporadically in Europe and North America. 1,5 Although endemic and sporadic Burkitt lymphoma are recognized by the World Health Organization as distinct entities, they are indistinguishable on histology.…”