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Treatment of Burkitt leukemia/lymphoma in adults

Arnold S Freedman, MD
Jonathan W Friedberg, MD
Section Editors
Andrew Lister, MD, FRCP, FRCPath, FRCR
Julie R Park, MD
Deputy Editor
Alan G Rosmarin, MD


Burkitt lymphoma (BL) is a highly aggressive B cell neoplasm characterized by the translocation and deregulation of the MYC gene on chromosome 8. Three distinct clinical forms of BL are recognized: endemic, sporadic, and immunodeficiency-associated. Although these differ in their epidemiology, clinical presentation, and genetic features, they are histologically identical and have similar clinical behavior. All three clinical forms are generally treated in a similar fashion.

BL and Burkitt leukemia are classified as different manifestations of the same disease. Diagnostic criteria have evolved to incorporate immunohistochemical, cytogenetic, and molecular diagnostic techniques. As such, many older studies included a heterogeneous patient population that contained many patients that would not be diagnosed with BL by current criteria.

The treatment of BL is presented here. The epidemiology, clinical presentation, and genetic features are discussed separately. (See "Epidemiology, clinical manifestations, pathologic features, and diagnosis of Burkitt lymphoma".)

Oncologic emergencies and treatment-related hematologic toxicities are common in the highly aggressive NHLs. Physicians must always be alert to their potential presence, and be prepared to deal with them urgently and effectively. In particular, patients with BL are at high risk for the development of tumor lysis syndrome (TLS). Prophylactic therapy for and management of TLS is presented in detail separately. (See "Tumor lysis syndrome: Definition, pathogenesis, clinical manifestations, etiology and risk factors" and "Clinical presentation and diagnosis of non-Hodgkin lymphoma", section on 'Oncologic emergencies'.)


The pretreatment evaluation both determines the bulk of disease and provides information about the individual's comorbidities that are likely to have an impact on treatment options. In addition to a history and physical examination, it is our practice to perform the following pretreatment studies in patients with BL:

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Literature review current through: Nov 2017. | This topic last updated: Nov 01, 2017.
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  1. Bishop PC, Rao VK, Wilson WH. Burkitt's lymphoma: molecular pathogenesis and treatment. Cancer Invest 2000; 18:574.
  2. Smeland S, Blystad AK, Kvaløy SO, et al. Treatment of Burkitt's/Burkitt-like lymphoma in adolescents and adults: a 20-year experience from the Norwegian Radium Hospital with the use of three successive regimens. Ann Oncol 2004; 15:1072.
  3. Hill QA, Owen RG. CNS prophylaxis in lymphoma: who to target and what therapy to use. Blood Rev 2006; 20:319.
  4. Sariban E, Edwards B, Janus C, Magrath I. Central nervous system involvement in American Burkitt's lymphoma. J Clin Oncol 1983; 1:677.
  5. Bernstein JI, Coleman CN, Strickler JG, et al. Combined modality therapy for adults with small noncleaved cell lymphoma (Burkitt's and non-Burkitt's types). J Clin Oncol 1986; 4:847.
  6. Patte C, Auperin A, Gerrard M, et al. Results of the randomized international FAB/LMB96 trial for intermediate risk B-cell non-Hodgkin lymphoma in children and adolescents: it is possible to reduce treatment for the early responding patients. Blood 2007; 109:2773.
  7. Cairo MS, Gerrard M, Sposto R, et al. Results of a randomized international study of high-risk central nervous system B non-Hodgkin lymphoma and B acute lymphoblastic leukemia in children and adolescents. Blood 2007; 109:2736.
  8. Kantarjian HM, O'Brien S, Smith TL, et al. Results of treatment with hyper-CVAD, a dose-intensive regimen, in adult acute lymphocytic leukemia. J Clin Oncol 2000; 18:547.
  9. 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.
  10. 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; 85:664.
  11. Cairo MS, Sposto R, Perkins SL, et al. Burkitt's and Burkitt-like lymphoma in children and adolescents: a review of the Children's Cancer Group experience. Br J Haematol 2003; 120:660.
  12. Spina M, Tirelli U, Zagonel V, et al. Burkitt's lymphoma in adults with and without human immunodeficiency virus infection: a single-institution clinicopathologic study of 75 patients. Cancer 1998; 82:766.
  13. 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.
  14. 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.
  15. Wang ES, Straus DJ, Teruya-Feldstein J, et al. Intensive chemotherapy with cyclophosphamide, doxorubicin, high-dose methotrexate/ifosfamide, etoposide, and high-dose cytarabine (CODOX-M/IVAC) for human immunodeficiency virus-associated Burkitt lymphoma. Cancer 2003; 98:1196.
  16. 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.
  17. Di Nicola M, Carlo-Stella C, Mariotti J, et al. High response rate and manageable toxicity with an intensive, short-term chemotherapy programme for Burkitt's lymphoma in adults. Br J Haematol 2004; 126:815.
  18. Blum KA, Lozanski G, Byrd JC. Adult Burkitt leukemia and lymphoma. Blood 2004; 104:3009.
  19. Kujawski LA, Longo WL, Williams EC, et al. A 5-drug regimen maximizing the dose of cyclophosphamide is effective therapy for adult Burkitt or Burkitt-like lymphomas. Cancer Invest 2007; 25:87.
  20. 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.
  21. 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.
  22. Cortes J, Thomas D, Rios A, et al. Hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone and highly active antiretroviral therapy for patients with acquired immunodeficiency syndrome-related Burkitt lymphoma/leukemia. Cancer 2002; 94:1492.
  23. 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.
  24. Hoelzer D, Walewski J, Döhner H, et al. Improved outcome of adult Burkitt lymphoma/leukemia with rituximab and chemotherapy: report of a large prospective multicenter trial. Blood 2014; 124:3870.
  25. Hoelzer D, Ludwig WD, Thiel E, et al. Improved outcome in adult B-cell acute lymphoblastic leukemia. Blood 1996; 87:495.
  26. Dunleavy K, Pittaluga S, Shovlin M, et al. Low-intensity therapy in adults with Burkitt's lymphoma. N Engl J Med 2013; 369:1915.
  27. Kelly JL, Toothaker SR, Ciminello L, et al. Outcomes of patients with Burkitt lymphoma older than age 40 treated with intensive chemotherapeutic regimens. Clin Lymphoma Myeloma 2009; 9:307.
  28. 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.
  29. Rizzieri DA, Johnson JL, Byrd JC, et al. Improved efficacy using rituximab and brief duration, high intensity chemotherapy with filgrastim support for Burkitt or aggressive lymphomas: cancer and Leukemia Group B study 10 002. Br J Haematol 2014; 165:102.
  30. Oriol A, Ribera JM, Bergua J, et al. High-dose chemotherapy and immunotherapy in adult Burkitt lymphoma: comparison of results in human immunodeficiency virus-infected and noninfected patients. Cancer 2008; 113:117.
  31. Corazzelli G, Frigeri F, Russo F, et al. RD-CODOX-M/IVAC with rituximab and intrathecal liposomal cytarabine in adult Burkitt lymphoma and 'unclassifiable' highly aggressive B-cell lymphoma. Br J Haematol 2012; 156:234.
  32. Ribera JM, García O, Grande C, et al. Dose-intensive chemotherapy including rituximab in Burkitt's leukemia or lymphoma regardless of human immunodeficiency virus infection status: final results of a phase 2 study (Burkimab). Cancer 2013; 119:1660.
  33. Evens AM, Carson KR, Kolesar J, et al. A multicenter phase II study incorporating high-dose rituximab and liposomal doxorubicin into the CODOX-M/IVAC regimen for untreated Burkitt's lymphoma. Ann Oncol 2013; 24:3076.
  34. Ribrag V, Koscielny S, Bosq J, et al. Rituximab and dose-dense chemotherapy for adults with Burkitt's lymphoma: a randomised, controlled, open-label, phase 3 trial. Lancet 2016.
  35. Thieblemont C, Coiffier B. Lymphoma in older patients. J Clin Oncol 2007; 25:1916.
  36. Romaguera JE, Fayad L, Rodriguez MA, et al. High rate of durable remissions after treatment of newly diagnosed aggressive mantle-cell lymphoma with rituximab plus hyper-CVAD alternating with rituximab plus high-dose methotrexate and cytarabine. J Clin Oncol 2005; 23:7013.
  37. Brenner DJ, Hall EJ. Computed tomography--an increasing source of radiation exposure. N Engl J Med 2007; 357:2277.
  38. Sodickson A, Baeyens PF, Andriole KP, et al. Recurrent CT, cumulative radiation exposure, and associated radiation-induced cancer risks from CT of adults. Radiology 2009; 251:175.
  39. Berrington de González A, Mahesh M, Kim KP, et al. Projected cancer risks from computed tomographic scans performed in the United States in 2007. Arch Intern Med 2009; 169:2071.
  40. Costa LJ, Xavier AC, Wahlquist AE, Hill EG. Trends in survival of patients with Burkitt lymphoma/leukemia in the USA: an analysis of 3691 cases. Blood 2013; 121:4861.