What is the role of chemotherapy in the treatment of Burkitt lymphoma/Burkitt-like lymphoma (BL/BLL)?

Updated: Dec 20, 2019
  • Author: Ali H Kanbar, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Intensive systemic chemotherapy is the treatment of choice for this aggressive disease in all its stages. [33] All clinical variants of Burkitt lymphoma are treated generally the same. The overall survival rate associated with Burkitt lymphoma depends upon the stage of the disease at initial diagnosis. Patients with localized disease respond well to chemotherapy and have an excellent survival rate. Patients with disseminated disease respond less well to chemotherapy and have a less favorable survival rate. Increasing age has also been associated with inferior outcome in most clinical trials. [6, 54] (See Staging and Prognosis.)

For patients who refuse, or are not candidates for clinical trials, short-duration, intensive, alkylator-based, multiagent chemotherapy regimens with adequate central nervous system (CNS) prophylaxis are necessary. Administration of less intensive chemotherapy regimens used in other non-Hodgkin lymphomas (NHL) (eg, CHOP [cyclophosphamide, hydroxydaunorubicin hydrochloride (doxorubicin hydrochloride), vincristine and prednisone]) usually results in frequent relapses and inferior survival. Of particular importance is the rapid administration of successive cycles of intensive multidrug therapy to prevent tumor regrowth. Dose reduction should also be avoided if possible. [55]

Most adult Burkitt lymphoma regimens were initially adopted from the pediatric study protocols that used several known active agents, including cyclophosphamide, vincristine, methotrexate, doxorubicin, and cytarabine. The French (LMB 81, 84, 86, and 89) and the German (B-NHL 83, B-NHL 86) protocols as well as the CODOX-M/IVAC regimen (cyclophosphamide, vincristine, doxorubicin, high-dose methotrexate / ifosfamide, etoposide, high-dose cytarabine) were modified and used in adult patients with acceptable outcomes (2-y overall survival: 40-74%). Other protocols (hyper-CVAD [modified fractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone], Cancer and Leukemia Group B [CALGB] 9251, etc) were evaluated primarily in adults.

A study by Todeschini et al found that intensive pediatric-based chemotherapy regimen increased remission and survival rates in both children and adults with Burtkitt lymphoma. [56]

Even though a standard regimen is not available yet, in general, three treatment approaches are available:

  • Intensive, short-duration regimens like CODOX-M/IVAC (Magrath regimen) and the CALGB 9251 protocol

  • Long-duration chemotherapy similar to acute lymphoblastic leukemia (ALL) treatment, like hyper-CVAD and the CALGB 8811 protocol

  • Combination regimens followed by autologous stem cell transplantation (SCT)

  • Most current regimens have added rituximab to previously established chemotherapy regimens

Despite the fact that no direct comparison has been done among these different approaches, the short-duration, more intense regimens are usually preferred in most US institutions, because they are faster to administer (ALL-type treatment may take up to 2 y and usually involves a maintenance arm) and less complicated than ALL-type treatment or SCT. The regimen most frequently used is CODOX-M/IVAC.


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