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1676 Part XI: Malignant Lymphoid Diseases Chapter 102: Burkitt Lymphoma 1677

compared with historical controls. 58–60 Based upon these findings and chemotherapy and considered for allogeneic stem cell transplantation
extrapolating from studies in other histologies of lymphoma, rituximab because autologous transplantation is often not beneficial in these
should be routinely incorporated in the treatment algorithms of BL. patients. 69
The NCI has published an experience using a lower-intensity che-
motherapy program consisting of infusional etoposide, vincristine and REFERENCES
doxorubicin, with bolus rituximab, cyclophosphamide and steroids
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mab resulted in an overall survival of 89 percent with only one death. 63,64 7. de-Thé G, Geser A, Day NE, et al: Epidemiological evidence for causal relationship
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duration regimen consisting of cyclophosphamide, ifosfamide, methotrex- 8. Magrath I: The pathogenesis of Burkitt’s lymphoma. Adv Cancer Res 55:133–270, 1990.
ate, vincristine, cytarabine, etoposide and glucocorticoids. This regimen 9. Kalter SP, Riggs SA, Cabanillas F, et al: Aggressive non-Hodgkin’s lymphomas in immu-
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a median age of 43 years, with 74 percent event-free survival at 3 years. 65 10. Manolov G, Manolova Y: Marker band in one chromosome 14 from Burkitt lympho-
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No advantage of autologous stem cell transplantation in patients 11. Zech L, Haglund U, Nilsson K, Klein G: Characteristic chromosomal abnormalities in
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mas. Int J Cancer 17:47–56, 1976.
transplantation into the initial therapeutic algorithm has been used 12. Dalla-Favera R, Bregni M, Erikson J, et al: Human c-myc onc gene is located on the
with reasonable results. 66 region of chromosome 8 that is translocated in Burkitt lymphoma cells. Proc Natl Acad
Historically, patients with HIV have been managed with Sci U S A 79:7824–7827, 1982.
less-intensive chemotherapy because of the concern of immunosup- 13. Taub R, Kirsch I, Morton C, et al: Translocation of the c-myc gene into the immuno-
globulin heavy chain locus in human Burkitt lymphoma and murine plasmacytoma
pression-related morbidity. In the highly active antiretroviral therapy cells. Proc Natl Acad Sci U S A 79:7837–7841, 1982.
(HAART) era, HIV-positive patients with BL should be treated simi- 14. Harris NL, Horning SJ: Burkitt’s lymphoma—The message from microarrays. N Engl J
larly to nonimmunocompromised patients, and the dose-adjusted Med 354:2495–2498, 2006.
R-EPOCH regimen may be an excellent option for these patients. The 15. Campo E: New pathogenic mechanisms in Burkitt lymphoma. Nat Genet 44:1288–1289, 2012.
16. Orem J, Mbidde EK, Lambert B, et al: Burkitt’s lymphoma in Africa, a review of the
rate of treatment failure in HIV-positive patients with BL was signifi- epidemiology and etiology. Afr Health Sci 7:166–175, 2007.
cantly lower with a highly aggressive protocol when compared with 17. Geser A, Brubaker G, Draper CC: Effect of a malaria suppression program on the inci-
dence of African Burkitt’s lymphoma. Am J Epidemiol 129:740–752, 1989.
patients who were treated with less-aggressive chemotherapy. In addi- 18. Mbulaiteye SM, Anderson WF, Bhatia K, et al: Trimodal age-specific incidence patterns
tion, patients tolerated the aggressive protocol reasonably well, particu- for Burkitt lymphoma in the United States, 1973–2005. Int J Cancer 126:1732–1739,
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19. Dorsett Y, Robbiani DF, Jankovic M, et al: A role for AID in chromosome translocations
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COURSE AND PROGNOSIS 20. Bhatia K, Huppi K, Spangler G, et al: Point mutations in the c-Myc transactivation
domain are common in Burkitt’s lymphoma and mouse plasmacytomas. Nat Genet
5:56–61, 1993.
To better define optimal therapy and outcome of adults with BL, an 21. Dave SS, Fu K, Wright GW, et al: Lymphoma/Leukemia Molecular Profiling Project:
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international effort focused on with a group of adult patients with BL. Molecular diagnosis of Burkitt’s lymphoma. N Engl J Med 354:2431–2442, 2006.
Authors of 12 large treatment series (10 prospective; two retrospective) 22. Hummel M, Bentink S, Berger H, et al; Molecular Mechanisms in Malignant Lym-
provided outcome information of patients enrolled on their clinical trials phomas Network Project of the Deutsche Krebshilfe: A biologic definition of Burkitt’s
lymphoma from transcriptional and genomic profiling. N Engl J Med 354:2419–2430,
who were older than age 40 years. In this pooled analysis, patients older 2006.
than age 40 years were underrepresented in the published literature, and 23. Love C, Sun Z, Jima D, et al: The genetic landscape of mutations in Burkitt lymphoma.
had significantly inferior outcomes in 10 of the 12 series. Despite this, Nat Genet 44:1321–1325, 2012.
the majority of adult patients were cured using these regimens. 24. Richter J, Schlesner M, Hoffmann S, et al: ICGC MMML-Seq Project: Recurrent muta-
tion of the ID3 gene in Burkitt lymphoma identified by integrated genome, exome and
According to data from the NCI’s Surveillance, Epidemiology, and transcriptome sequencing. Nat Genet 44:1316–1320, 2012.
End Results (SEER) program, up to 30 percent of BL diagnosis in the 25. Schmitz R, Young RM, Ceribelli M, et al: Burkitt lymphoma pathogenesis and thera-
peutic targets from structural and functional genomics. Nature 490:116–120, 2012.
United States includes an “older” group of patients, age 60 years or older. 26. Biggar RJ, Chaturvedi AK, Goedert JJ, Engels EA: HIV/AIDS Cancer Match Study:
Treatment options may be limited for this group of patients, as many AIDS-related cancer and severity of immunosuppression in persons with AIDS. J Natl
older patients may not tolerate high-dose methotrexate and are not can- Cancer Inst 99:962–972, 2007.
didates for autologous transplantation. Although the number of older 27. Aka P, Vila MC, Jariwala A, et al: Endemic Burkitt lymphoma is associated with
strength and diversity of Plasmodium falciparum malaria stage-specific antigen anti-
patients on the dose adjusted R-EPOCH trial was limited, this regimen body response. Blood 122:629–635, 2013.
has demonstrated safety in elderly patients with DLBCL. A confirmatory 28. Mannucci S, Luzzi A, Carugi A, et al: EBV reactivation and chromosomal polysomies:
trial conducted in several centers is ongoing and enrolling older patients. Euphorbia tirucalli as a possible cofactor in endemic Burkitt lymphoma. Adv Hematol
2012:149780, 2012.
For patients with relapsed or refractory disease, autologous trans- 29. Rochford R, Cannon MJ, Moormann AM: Endemic Burkitt’s lymphoma: A polymicro-
plantation is best reserved for patients inadequately treated initially, as bial disease? Nat Rev Microbiol 3:182–187, 2005.
a consolidation procedure. Patients who relapse after appropriate ther- 30. Thorley-Lawson DA, Allday MJ: The curious case of the tumour virus: 50 years of
Burkitt’s lymphoma. Nat Rev Microbiol 6:913–924, 2008.
apy for BL tend to have highly treatment-resistant disease, with almost 31. Molyneux EM, Rochford R, Griffin B, et al: Burkitt’s lymphoma. Lancet 379:1234–1244,
uniformly unfavorable outcome. These patients can be retreated with 2012.

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