Burkitt’s lymphoma: pathophysiology, diagnostics and treatment

Authors: L. Šmardová 1;  I. Vášová 1;  M. Navrátil 1;  Z. Křenová 2;  D. Šálek 1;  A. Marečková 1;  L. Křen 3;  K. Bolčák 4;  J. Vorlíček 1;  J. Mayer 1
Authors‘ workplace: Interní hematoonkologická klinika Fakultní nemocnice Brno a lékařské fakulty Masarykovy univerzity, Brno, 2Klinika dětské onkologie Fakultní nemocnice Brno a lékařské fakulty Masarykovy univerzity, Brno, 3Ústav patologie Fakultní nemocnice Brno a lékařské 1
Published in: Transfuze Hematol. dnes,14, 2008, No. 3, p. 131-139.
Category: Comprehensive Reports, Original Papers, Case Reports


Burkitt’s lymphoma is a highly aggressive mature B-cell malignancy with a characteristic chromosomal translocation of c-myc proto-oncogene. Clinically, it manifests mostly by an extranodal involvement, affection of gastrointestinal system and usually by developing of a big tumor mass. Exactly 50 years ago Denis Burkitt, an Irish doctor of medicine, described as the first the endemic variant of this lymphoma in children in eastern Africa and his cooperation with virologist Anthony Epstein led to the discovery of the virus etiology of this lymphoma. Burkitt’s lymphoma requires an early medical treatment that is nowadays based on intensive combined cycles of chemotherapeutic agents. A part of the therapy includes a prophylaxis of the affection of central nervous system by tumor cells, where high-dose methotrexate, cytosinarabinosid and intrathecal therapy is used. With respect to regular CD20 positivity in cancer cells, the best curative outcome is achieved by a combination of cytostatic agents therapy with a monoclonal antibody therapy rituximab. Burkitt’s lymphoma is nowadays ranked among potentially curable diseases.

Key words:
Burkitt’s lymphoma, Epstein-Barr virus, molecular biology, c-myc, tumor lysis syndrom, chemotherapy


1. Burkitt D. A sarcoma involving the jaws in African children. Br J Surg 1958; 46(197): 218–223.

2. Burkitt D. Determining the climatic limitations of a children’s cancer common in Africa. Br Med J 1962; 2(5311): 1019–1023.

3. Epstein MA, Achong BG, Barr YM. Virus particles in cultured lymphoblasts from Burkitt’s lymphoma. Lancet 1964; 1: 702–703.

4. Coakley D. Denis Burkitt and his contribution to haematology/oncology. Br J Haematol 2006; 135: 17–25.

5. Kelly GL, Rickinson AB. Burkitt lymphoma: revisiting the pathogenesis of a virus-associated malignancy. Haematol 2007; 2007: 277–284.

6. Diebold J, Jaffe ES, Raphael M. Burkitt lymphoma. In: Jaffe ES, Harris NL, Stein H, et al. World Health Organization Classification of Tumours. Pathology and genetics of tumours of haematopoietic and lymphoid tissues. IARC Press, Lyon 2001; 181–184.

7. Troxell ML, Bangs CD, Cherry AM, et al. Cytologic diagnosis of Burkitt lymphoma. Cancer 2005; 105(5): 310–318.

8. Stein H, Hummel M. Burkitt’s and Burkitt-like lymphoma. Molecular definition and value of the World Health Organisation’s diagnostic criteria. Pathologe 2007; 28(1): 41–45.

9. Cogliatti SB, Novak U, Henz S, et al. Diagnosis of Burkitt lymphoma in due time: a practical approach. Br J Haematol 2006; 134: 294–301.

10. Manolov G, Manolova Y. Marker band in one chromosome 14 from Burkitt lymphomas. Nature 1972; 237(5349): 33–44.

11. Zech L, Haglund U, Nilsson K, et al. Characteristic chromosomal abnormalities in biopsies and lymphoid-cell lines from patients with Burkitt and non-Burkitt lymphomas. Int J Cancer 1976; 17(1): 47–56.

12. Dalla-Favera R, Bregni M, Erikson J, et al. Human c-myc onc gene is located on the region of chromosome 8 that is translocated in Burkitt lymphoma cells. Proc Natl Acad Sci USA 1982; 79(24): 7824–7827.

13. Onciu M, Schlette E, Zhou Y, et al. Secondary chromosomal abnormalities predict outcome in pediatric and adult high-stage Burkitt lymphoma. Cancer 2006; 107(5): 1084–1092.

14. Macpherson N, Lesack D, Klasa R, et al. Small noncleaved, non-Burkitt’s (Burkitt like) lymphoma: Cytogenetics predicts outcome and reflect clinical presentation. J Clin Oncol 1999; 17(5): 1558–1567.

15. Wilda M, Bruch J, Nardet L, et al. Inactivation of the ARF-MDM-2-p53 pathway in sporadic Burkitt’s lymphoma in children. Leukemia 2004; 18: 584-588.

16. Hummel M, Bentink S, Berger H, et al. A biologic definition of Burkitt’s lymphoma from transcriptional and genomic profiling. N Engl J Med 2006; 354(23): 2419–2430.

17. Dave SS, Fu K, Wright GW, et al. Molecular diagnosis of Burkitt’s lymphoma. N Engl J Med 2006; 354(23): 2431–2442.

18. Keller C, Savage DG, Rusta-Villa M, et al. Primary Burkitt lymphoma of the uterine corpus. Leuk Lymphoma 2006; 47(1): 141–5.

19. Ferry JA. Burkitt’s lymphoma: clinicopathologic features and differential diagnosis. Oncologist 2006; 11(4): 375–383.

20. Murphy SB. Classification, staging and end results of treatment of childhood non-Hodgkin’s lymphomas: dissimilarities from lymphoma in adults. Semin Oncol 1980; 7: 322–329.

21. National Comprehensive Cancer Network, Practice guidelines in Oncology, version 3.2007.

22. Cohen LF, Balow JE, Magrath IT, et al. Acute tumor lysis syndrome. A review of 37 patients with Burkitt’s lymphoma. Am J Med 1980; 68: 486–491.

23. Belada D, Trněný M, et al. Diagnostické a léčebné postupy u nemocných s maligními lymfomy II. 3. vyd. Praha, 2007; 33–35.

24. Yustein JT, Dang V. Biology and treatment of Burkitt’s lymphoma. Curr Opin Hematol 2007; 14: 375–381.

25. Blum KA, Lozanski G, Byrd JC. Adult Burkitt leukemia and lymphoma. Blood 2004; 104(10): 3009–3020.

26. Magrath IT, Haddy TB, Adde MA. Treatment of patients with high grade non-Hodgkin’s lymphomas and central nervous system involvement: is radiation an essential component of therapy? Leuk Lymphoma 1996; 21(1–2): 99–105.

27. Soussain C, Patte C, Ostronoff M, et al. Small noncleaved cell lymphoma and leukemia in adults: a retrospective study of 65 adults treated with the LMB pediatric protocols. Blood 1995; 85(3): 664–674.

28. Magrath I, Adde M, Shad A, et al. Adults and children with small non-cleaved-cell lymphoma have a similar excellent outcome when treated with the same chemotherapy regimen. J Clin Oncol 1996; 14(3): 925–934.

29. Todeschini G, Tecchio C, Degani D, et al. Eighty-one percent event-free survival in advanced Burkitt’s lymphoma/leukemia: no differences in outcome between pediatric and adult patients treated with the same intensive pediatric protocol. Ann Oncol 1997; 8 (Suppl 1): 77–81.

30. Longo DL, Duffey PL, Jaffe ES, et al. Diffuse small noncleaved-cell, non-Burkitt’s lymphoma in adults: a high-grade lymphoma responsive to ProMACE-based combination chemotherapy. J Clin Oncol 1994; 12(10): 2153–2159.

31. Diviné M, Casassus P, Koscielny S, et al. Burkitt lymphoma in adults: a prospective study of 72 patients treated with an adapted pediatric LMB protocol. Ann Oncol 2005; 16(12): 1928–1935.

32. Hoelzer D, Ludwig WD, Thiel E, et al. Improved outcome in adult B-cell acute lymphoblastic leukemia. Blood 1996; 87(2): 495–508.

33. Hoelzer D, Hiddemann W, Baumann A, et al. High cure rate of adult Burkitt’s and other high grade NHL by the combination of short intensive chemotherapy cycles with rituximab. Haematologica 2007; 92(S1): 151.

34. Mead GM, Sydes MR, Walewski J, et al. An international evaluation of CODOX-M and CODOX-M alternating with IVAC in adult Burkitt’s lymphoma: results of United Kingdom Lymphoma Group LY06 study. Ann Oncol 2002; 13(8): 1264–1274.

35. Thomas DA, Cortes J, O’Brien S, et al. Hyper-CVAD program in Burkitt’s-type adult acute lymphoblastic leukemia. J Clin Oncol 1999; 17(8): 2461–2470.

36. Thomas DA, Faderl S, O’Brien S, et al. Chemoimmunotherapy with hyper-CVAD plus rituximab for the treatment of adult Burkitt and Burkitt-type lymphoma or acute lymphoblastic leukemia. Cancer 2006; 106(7): 1569–1580.

37. Boué F, Gabarre J, Gisselbrecht C, et al. Phase II trial of CHOP plus rituximab in patients with HIV-associated non-Hodgkin’s lymphoma. J Clin Oncol 2006; 24(25): 4123–4128.

38. Sweetenham JW, Pearce R, Taghipour G, et al. Adult Burkitt’s and Burkitt-like non-Hodgkin’s lymphoma - outcome for patients treated with high-dose therapy and autologous stem-cell transplantation in first remission or at relapse: results from the European Group for Blood and Marrow Transplantation. J Clin Oncol 1996; 14(9): 2465–2472.

39. Imhoff GW, Holt B, MacKenzie MA, et al. Short intensive sequential therapy followed by autologous stem cell transplantation in adult Burkitt, Burkitt-like and lymphoblastic lymphoma. Leukemia 2005; 19(6): 945–952.

40. Song KW, Barnett MJ, Gascoyne RD, et al. Haematopoietic stem cell transplantation as primary therapy of sporadic adult Burkitt lymphoma. Br J Haematol 2006; 133(6): 634–637.

41. Woessmann W, Seidemann K, Mann G, et al. The impact of the methotrexate administration schedule and dose in the treatment of children and adolescents with B-cell neoplasms: a report of the BFM Group Study NHL-BFM95. Blood 2005; 105(3): 948–958.

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