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Burkitt Lymphoma and Atypical Burkitt or Burkitt-like Lymphoma: Should These be Treated as Different Diseases?

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Abstract

Burkitt lymphoma (BL) is a mature B-cell non-Hodgkin lymphoma with an aggressive clinical course. Since the advent of short, intensive, multiagent chemoimmunotherapy regimens, it has carried a favorable prognosis. BL has been rather well characterized, whereas the other lymphomas morphologically resembling it are more heterogeneous. The cases classified as atypical BL/Burkitt-like lymphoma by the 2001 World Health Organization (WHO) Classification of Tumors of Hematopoietic and Lymphoid Tissue were thought to represent a continuum between BL and diffuse large B-cell lymphoma (DLBCL). The optimal therapeutic strategy for this provisional entity was not definitively established. However, recent incorporation of molecular genetic data into the 2008 WHO Classification has allowed further refinements with significant therapeutic implications, including the designation of a new provisional entity, “B-cell lymphoma, unclassifiable, with features intermediate between BL and DLBCL.” This review presents a comprehensive overview of the previously designated provisional entity of atypical BL/BLL in conjunction with a detailed comparison with BL and DLBCL.

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References

Papers of particular interest, published recently, have been highlighted as:• Of importance

  1. Diebold JE, Jaffe ES, Raphael M, Warnke RA. Burkitt lymphoma. In: Jaffe ES, Harris NL, Stein H, Vardiman J, eds. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, France: IARC Press; 2001:181–184

    Google Scholar 

  2. Jaffe ES. The 2008 WHO classification of lymphomas: implications for clinical practice and translational research. Hematology Am Soc Hematol Educ Program. 2009:523–531.

    Google Scholar 

  3. Burkitt DP. The discovery of Burkitt’s lymphoma. Cancer. 1983;51:1777–1786.

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  5. Kutok JL, Wang F. Spectrum of Epstein-Barr virus-associated diseases. Annu Rev Pathol. 2006;1:375–404.

    Article  CAS  PubMed  Google Scholar 

  6. Hecht JL, Aster JC. Molecular biology of Burkitt’s lymphoma. J Clin Oncol. 2000;18:3707–3721.

    CAS  PubMed  Google Scholar 

  7. Burmeister T, Schwartz S, Horst HA, et al. Molecular heterogeneity of sporadic adult Burkitt-type leukemia/lymphoma as revealed by PCR and cytogenetics: correlation with morphology, immunology and clinical features. Leukemia. 2005;19:1391–1398.

    Article  CAS  PubMed  Google Scholar 

  8. Neri A, Barriga F, Knowles DM, Magrath IT, Dalla-Favera R. Different regions of the immunoglobulin heavy-chain locus are involved in chromosomal translocations in distinct pathogenetic forms of Burkitt lymphoma. Proc Natl Acad Sci U S A. 1988;85:2748–2752.

    Article  CAS  PubMed  Google Scholar 

  9. Klapproth K, Wirth T. Advances in the understanding of MYC-induced lymphomagenesis. Br J Haematol. 2010;149:484–497.

    Article  CAS  PubMed  Google Scholar 

  10. • Mead GM, Barrans SL, Qian W, et al. A prospective clinicopathologic study of dose-modified CODOX-M/IVAC in patients with sporadic Burkitt lymphoma defined using cytogenetic and immunophenotypic criteria (MRC/NCRI LY10 trial). Blood. 2008;112:2248–2260. This is one of the few prospective clinicopathologic studies of aggressive, mature B-cell NHL treated uniformly with a similar risk-adapted therapeutic regimen.

    Article  CAS  PubMed  Google Scholar 

  11. McClure RF, Remstein ED, Macon WR, et al. Adult B-cell lymphomas with Burkitt-like morphology are phenotypically and genotypically heterogeneous with aggressive clinical behavior. Am J Surg Pathol. 2005;29:1652–1660.

    Article  PubMed  Google Scholar 

  12. Mossafa H, Damotte D, Jenabian A, et al. Non-Hodgkin’s lymphomas with Burkitt-like cells are associated with c-Myc amplification and poor prognosis. Leuk Lymphoma. 2006;47:1885–1893.

    Article  CAS  PubMed  Google Scholar 

  13. Au WY, Horsman DE, Gascoyne RD, Viswanatha DS, Klasa RJ, Connors JM. The spectrum of lymphoma with 8q24 aberrations: a clinical, pathological and cytogenetic study of 87 consecutive cases. Leuk Lymphoma. 2004;45:519–528.

    Article  CAS  PubMed  Google Scholar 

  14. • Barrans S, Crouch S, Smith A, et al. Rearrangement of MYC is associated with poor prognosis in patients with diffuse large B-cell lymphoma treated in the era of rituximab. J Clin Oncol. 2010;28:3360–3365. MYC rearrangement was determined to be an independent factor for worse survival (in conjunction with age and International Prognostic Index) for de novo DLBCL treated with CHOP-R.

    Article  CAS  PubMed  Google Scholar 

  15. A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin’s lymphoma. The Non-Hodgkin’s Lymphoma Classification Project. Blood. 1997;89:3909–3918.

    Google Scholar 

  16. Harris NL, Jaffe ES, Stein H, et al. A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood. 1994;84:1361–1392.

    CAS  PubMed  Google Scholar 

  17. National Cancer Institute sponsored study of classifications of non-Hodgkin’s lymphomas: Summary and description of a working formulation for clinical usage. The Non-Hodgkin’s Lymphoma Pathologic Classification Project. Cancer. 1982;49:2112–2135.

    Google Scholar 

  18. Braziel RM, Arber DA, Slovak ML, et al. The Burkitt-like lymphomas: a Southwest Oncology Group study delineating phenotypic, genotypic, and clinical features. Blood. 2001;97:3713–3720.

    Article  CAS  PubMed  Google Scholar 

  19. Haralambieva E, Boerma EJ, van Imhoff GW, et al. Clinical, immunophenotypic, and genetic analysis of adult lymphomas with morphologic features of Burkitt lymphoma. Am J Surg Pathol. 2005;29:1086–1094.

    PubMed  Google Scholar 

  20. • LaCasce AS, Kho ME, Friedberg JW, et al. Comparison of referring and final pathology for patients with non-Hodgkin’s lymphoma in the National Comprehensive Cancer Network. J Clin Oncol. 2008;26:5107–5112. The overall pathological discordance rate for five B-cell NHL subtypes was 6% for 731 cases; the highest concordance rate was for DLBCL. Therapy would have been altered for 80% of the discordant cases.

    Article  PubMed  Google Scholar 

  21. Gormley RP, Madan R, Dulau AE, et al. Germinal center and activated b-cell profiles separate Burkitt lymphoma and diffuse large B-cell lymphoma in AIDS and non-AIDS cases. Am J Clin Pathol. 2005;124:790–798.

    Article  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  23. 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;354:2419–2430.

    Article  CAS  PubMed  Google Scholar 

  24. Klapper W, Szczepanowski M, Burkhardt B, et al. Molecular profiling of pediatric mature B-cell lymphoma treated in population-based prospective clinical trials. Blood. 2008;112:1374–1381.

    Article  CAS  PubMed  Google Scholar 

  25. 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;14:925–934.

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  28. Lee EJ, Petroni GR, Schiffer CA, et al. Brief-duration high-intensity chemotherapy for patients with small noncleaved-cell lymphoma or FAB L3 acute lymphocytic leukemia: results of cancer and leukemia group B study 9251. J Clin Oncol. 2001;19:4014–4022.

    CAS  PubMed  Google Scholar 

  29. 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;13:1264–1274.

    Article  CAS  PubMed  Google Scholar 

  30. Rizzieri DA, Johnson JL, Niedzwiecki D, et al. Intensive chemotherapy with and without cranial radiation for Burkitt leukemia and lymphoma: final results of Cancer and Leukemia Group B Study 9251. Cancer. 2004;100:1438–1448.

    Article  CAS  PubMed  Google Scholar 

  31. Lacasce A, Howard O, Lib S, et al. Modified Magrath regimens for adults with Burkitt and Burkitt-like lymphomas: preserved efficacy with decreased toxicity. Leuk Lymphoma. 2004;45:761–767.

    Article  CAS  PubMed  Google Scholar 

  32. 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;106:1569–1580.

    Article  CAS  PubMed  Google Scholar 

  33. Dunleavy K, Healey Bird BR, Pittaluga S, et al. Efficacy and toxicity of dose-adjusted EPOCH-rituximab in adults with newly diagnosed Burkitt lymphoma [abstract 8035]. J Clin Oncol. 2007;25:449 s.

    Article  Google Scholar 

  34. Hoelzer D, Hiddemann W, Baumann A, et al. High survival rate in adult Burkitt’s lymphoma/leukemia and diffuse large B-cell lymphoma with mediastinal involvement [abstract 518]. Blood. 2007;110:159a.

    Google Scholar 

  35. • Hoelzer D. Recent results in the treatment of Burkitt lymphomas. Ann Oncol. 2008;19(suppl 4):iv83. Outcomes were improved with the addition of rituximab to the Berlin-Frankfurt-Munster (BFM) regimens, but there was a high incidence of central nervous system relapse in older patients with Burkitt lymphoma/leukemia owing to reduced systemic methotrexate and omission of systemic cytarabine.

    Google Scholar 

  36. Abramson JS, Barnes JA, Toomey CE, et al. Rituximab added to CODOX-M/IVAC is highly effective in HIV-negative and HIV-positive Burkitt lymphoma [abstract 3595]. Blood. 2008;112.

  37. Kenkre VP, Stock W. Burkitt lymphoma/leukemia: improving prognosis. Clin Lymphoma Myeloma. 2009;9 Suppl 3:S231–238.

    PubMed  Google Scholar 

  38. Nomura Y, Karube K, Suzuki R, et al. High-grade mature B-cell lymphoma with Burkitt-like morphology: results of a clinicopathological study of 72 Japanese patients. Cancer Sci. 2008;99:246–252.

    Article  CAS  PubMed  Google Scholar 

  39. Carbone A, Gloghini A, Aiello A, Testi A, Cabras A. B-cell lymphomas with features intermediate between distinct pathologic entities. From pathogenesis to pathology. Hum Pathol. 2010;41:621–631.

    Article  CAS  PubMed  Google Scholar 

  40. Bellan C, Stefano L, Giulia de F, Rogena EA, Lorenzo L. Burkitt lymphoma versus diffuse large B-cell lymphoma: a practical approach. Hematol Oncol. 2009;27:182–185.

    Article  PubMed  Google Scholar 

  41. Hasserjian RP, Ott G, Elenitoba-Johnson KS, Balague-Ponz O, de Jong D, de Leval L. Commentary on the WHO classification of tumors of lymphoid tissues (2008): “Gray zone” lymphomas overlapping with Burkitt lymphoma or classical Hodgkin lymphoma. J Hematop. 2009:e-published.

  42. Karsan A, Gascoyne RD, Coupland RW, Shepherd JD, Phillips GL, Horsman DE. Combination of t(14;18) and a Burkitt’s type translocation in B-cell malignancies. Leuk Lymphoma. 1993;10:433–441.

    Article  CAS  PubMed  Google Scholar 

  43. Kanungo A, Medeiros LJ, Abruzzo LV, Lin P. Lymphoid neoplasms associated with concurrent t(14;18) and 8q24/c-MYC translocation generally have a poor prognosis. Mod Pathol. 2006;19:25–33.

    Article  CAS  PubMed  Google Scholar 

  44. Lin P, Medeiros LJ. High-grade B-cell lymphoma/leukemia associated with t(14;18) and 8q24/MYC rearrangement: a neoplasm of germinal center immunophenotype with poor prognosis. Haematologica. 2007;92:1297–1301.

    Article  CAS  PubMed  Google Scholar 

  45. Snuderl M, Kolman OK, Chen YB, et al. B-cell lymphomas with concurrent IGH-BCL2 and MYC rearrangements are aggressive neoplasms with clinical and pathologic features distinct from Burkitt lymphoma and diffuse large B-cell lymphoma. Am J Surg Pathol. 2010;34:327–340.

    Article  PubMed  Google Scholar 

  46. Wu D, Wood BL, Dorer R, Fromm JR. “Double-Hit” mature B-cell lymphomas show a common immunophenotype by flow cytometry that includes decreased CD20 expression. Am J Clin Pathol. 2010;134:258–265.

    Article  PubMed  Google Scholar 

  47. Keller CE, Nandula S, Fisher J, et al. The spectrum of B-cell non-Hodgkin lymphomas with dual IgH-BCL2 and BCL6 translocations. Am J Clin Pathol. 2008;130:193–201.

    Article  CAS  PubMed  Google Scholar 

  48. van Imhoff GW, Boerma EJ, van der Holt B, et al. Prognostic impact of germinal center-associated proteins and chromosomal breakpoints in poor-risk diffuse large B-cell lymphoma. J Clin Oncol. 2006;24:4135–4142.

    Article  PubMed  Google Scholar 

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Thomas, D.A., O’Brien, S., Faderl, S. et al. Burkitt Lymphoma and Atypical Burkitt or Burkitt-like Lymphoma: Should These be Treated as Different Diseases?. Curr Hematol Malig Rep 6, 58–66 (2011). https://doi.org/10.1007/s11899-010-0076-4

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  • DOI: https://doi.org/10.1007/s11899-010-0076-4

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