Burkitt’s lymphoma variant of post-transplant lymphoproliferative disease (PTLD)

Abstract

The occurrence of posttransplant lymphoproliferative disorder (PTLD) in solid organ allograft recipients can be quite varied in clinical presentation, histopathological characteristics and frequency. A variety of lymphomas can develop as a PTLD although some types appear infrequently and remain poorly understood in this clinical setting. In this report, we describe two cases of Burkitt s lymphoma presenting as a PTLD following liver transplantation. The recipients were 12 and 44 years of age and displayed gastrointestinal involvement by the tumors several years following transplant. The tumors displayed the typical histological features of Burkitt s lymphoma and were markedly positive for EBV. The tumors displayed similar immunophenotypic characteristics by flow cytometry and had rearrangements of the immunoglobulin J-H heavy chain. The tumors required aggressive chemotherapy and a cessation of immunosuppressive therapy. This report demonstrates that Burkitt s type lymphomas can develop in the posttransplant setting and that these tumors contain morphologic, cytofluorographic and molecular features identical to Burkitt s lymphomas that occur in non-transplant patients. Our experience is that these PTLD- Burkitt s lymphomas behave aggressively and require intensive chemotherapeutic intervention.

Post‐transplant Burkitt lymphoma is a more aggressive and distinct form of post‐transplant lymphoproliferative disorder

“…5,[9][10][11][12][13][14][15][16][17] This expression of EBV is much higher than that reported for both sporadic and immunodeficiencyassociated BL, primarily HIV-associated BL. [6][7][8] The interplay of active EBV infection in the immunosuppressed state with the development of BL-PTLD is noteworthy.…”

“…[6][7][8] However, previous patient series of BL occurring in the post-transplant setting have a higher incidence of EBV expression (>70%), greater than that reported for immunodeficiency-associated BL, and more akin to the endemic type and other PTLDs. [7][8][9][10][11][12][13][14][15][16][17] The aims of this study were to characterize the patients with BL-PTLD at our institution, a large pediatric transplant center, and to delineate what specific clinical, pathologic, and/or genetic aspects of this disease are discrete.…”

“…32 Our reported longer interval for BL-PTLD after transplant is similar to other published pediatric case reports for BL-PTLD, ranging from 24-72 months post-transplant. [9][10][11] One hypothesis for this longer lag time in BL-PTLD is that there is a more complex disease pathophysiology needed to induce the genetic dysregulation of the c-MYC oncogene that accumulates over time in contrast to the immediate cytotoxic T-lymphocyte immunosuppression and subsequent EBV B-cell proliferation of memory B-cells in other PTLDs. Five patients did have some type of PTLD proliferation (early EBV associated and P-PTLD) before the BL-PTLD presentation (Table 2), including 2 of the heart transplant patients (BL-3 and 9).…”

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“…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.…”

PTLD Burkitt Lymphoma in a Patient with Remote Lymphomatoid Granulomatosis

“…EBV is associated with high proportion (89%) of posttransplant B-cell PTLD [5] and has been shown to promote B-cell proliferation by expressing an EBV-transforming protein (latent membrane protein 1) that engages the tumor necrosis factor receptor signal-transduction pathway to activate important regulators of cell growth, differentiation, or survival, such as nuclear factor kappa B and mitogen-activated protein kinases [4]. In particular, pretransplant EBV seronegativity followed by posttransplant seroconversion has been shown to be a major contributing risk factor for developing PTLD [6–8]. Monitoring the viral loads during the treatment may aid management because persistently elevated titers necessitate alternative treatment [9].…”