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

Abstract

BACKGROUND: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.METHODS:Patients with BL, diagnosed in the post‐transplant setting, (patients aged ≤18 years) were retrieved from the pathology archives at Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center from 1982 to 2010. Clinical outcomes were obtained along with pathologic review.RESULTS:Twelve patients with pediatric BL in the post‐transplant setting (9 boys, 3 girls) were retrieved. The patients displayed a monomorphic population of small to intermediate‐sized, noncleaved, lymphoid elements with a “starry‐sky” pattern. The immunophenotype for patients available to the study was CD20+ (n = 9/10), CD10+ (n = 8/8), bcl‐6+ (n = 11/11), with a near 100% Ki‐67/MIB‐1 proliferation index (n = 7/7), and negative for TdT (n = 7/7). Most pretransplant Epstein‐Barr virus titers were negative (n = 8/10), with post‐transplant titers positive in all tested patients (n = 11), and with positive Epstein‐Barr–encoded RNA in situ hybridization in most cases (n = 9/11). The median time from transplantation to diagnosis was 52 months (range, 6‐107 months). Nine patients were currently alive after immediate antineoplastic chemotherapy, with median disease‐free time of 93 months from diagnosis (range, 2‐199 months).CONCLUSIONS:BL‐PTLD had a higher Epstein‐Barr virus incidence compared with sporadic and immunodeficiency‐associated BL and represented a distinct monomorphic PTLD. Although some M‐PTLDs can be managed less aggressively with decreased immunosuppression alone, immediate lymphoma‐specific chemotherapy was associated with a favorable outcome and was strongly recommended. Cancer 2011;. © 2011 American Cancer Society.

How I treat posttransplant lymphoproliferative disorders

“…Therefore, we performed a literature search and identified 6 small case series (3 pediatric and 3 adult) focusing on treatment of aggressive PTLD, including 39 patients with BL-PTLD. [58][59][60][61][62][63] Twenty-five of 26 evaluable cases (those with information on MYC) had a proven MYC rearrangement. In contrast to sporadic (30%) and HIV-associated BL (25%-40%), most cases (82%) were EBV associated.…”

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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 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.…”

“…5 Although most posttransplant lymphoproliferative disorders originate from postgerminal center cells, Burkitt lymphoma has a germinal center molecular profile and has more aggressive behavior than other posttransplant lymphoproliferative disorders. 6 Therefore, it is important to understand the clinicopathologic features of Burkitt lymphoma in transplant recipients.…”

Posttransplant Lymphoproliferative Disorders Following Solid Organ Transplantation

“…Burkitt lymphoma seems to respond well to immediate aggressive chemotherapy. (Picarsic et al, 2010) Hodgkin disease is often associated with EBV and responds well to standard therapy. (Bierman et al, 1996) Available literature suggests that T -cell PTLD is clinically aggressive and may be associated with a poor prognosis.…”