What is Burkitt lymphoma?

From the Guidelines

Burkitt lymphoma should be treated with intensive combination chemotherapy regimens that include rituximab, as this has been shown to improve outcomes in adult patients, particularly in terms of event-free survival and overall survival rates. The most recent and highest quality study, published in 2024 in the Annals of Oncology 1, supports the use of rituximab in combination with chemotherapy for the treatment of Burkitt lymphoma. This study found that the addition of rituximab to intensive chemotherapy regimens resulted in improved response rates, with complete remission rates of 83%-88%, and long-term event-free survival rates of 80%-89%.

Some key points to consider when treating Burkitt lymphoma include:

• The importance of immediate treatment due to the aggressive nature of the disease
• The need for central nervous system prophylaxis with intrathecal chemotherapy
• The use of intensive combination chemotherapy regimens, such as CODOX-M/IVAC or R-EPOCH, which include rituximab
• The consideration of patient age, disease stage, and overall health status when selecting a treatment regimen
• The potential for substantial toxicity associated with these regimens, particularly in high-risk patients

According to the study by the Group for Research on Adult ALL (GRAALL) and the Lymphoma Study Association (LYSA) study group, as cited in 1, rituximab was associated with significant improvements in 3-year event-free survival rate and 3-year overall survival rate. The Alliance Cancer and Leukemia Group B (CALGB) 10002 study, also cited in 1, found that high remission rates and durable remissions were achieved with rituximab-eChT, but noted that the regimen was associated with substantial toxicity.

Overall, the evidence supports the use of rituximab in combination with intensive chemotherapy for the treatment of Burkitt lymphoma, with the goal of improving outcomes and reducing morbidity and mortality.

From the Research

Overview of Burkitt Lymphoma

• Burkitt lymphoma (BL) is a highly aggressive B-cell non-Hodgkin lymphoma characterized by marked tumor proliferation resulting from translocation of the MYC oncogene 2.
• Distinct clinical variants include endemic, sporadic, and immunodeficiency-associated cases, all of which are characterized by rapidly dividing tumor masses that quickly disseminate to extranodal sites, including the bone marrow and central nervous system (CNS) 2.

Diagnosis and Treatment

• Prompt recognition and initiation of comprehensive supportive care are essential for prevention of early complications, such as tumor lysis syndrome and multisystem organ dysfunction 2.
• BL is highly sensitive to chemotherapy, and patients who tolerate highly intensive combination chemotherapy regimens are frequently cured 2, 3.
• The infusional regimen of dose-adjusted etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin, and regular- or double-dose rituximab (DA-EPOCH-R or -RR) is less toxic than standard BL regimens, yet maintains high rates of cure across a diverse range of patients 2.

Treatment Outcomes

• Approximately 50%-80% of adult patients with Burkitt's lymphoma or small, noncleaved lymphoma can be cured with intensive chemotherapy regimens 4.
• A very short, intensive pediatric chemotherapy program tested in adult Burkitt's lymphoma patients resulted in a complete response rate of 77% and an overall survival rate of 77% 5.
• A 5-drug regimen maximizing the dose of cyclophosphamide is effective therapy for adult Burkitt or Burkitt-like lymphomas, with a complete response rate of 91% and a 3-year event-free survival rate of 64% 6.

Challenges and Considerations

• The high proliferation rate of Burkitt's lymphoma enhances the risk for tumor lysis syndrome, which results from metabolic imbalances, such as hyperuricemia, that occur as large numbers of malignant cells are lysed during cytotoxic chemotherapy 4.
• The lifetime risk of developing central nervous system disease is 20%-30% for Burkitt's lymphoma, and all chemotherapy regimens with activity in Burkitt's lymphoma utilize some form of central nervous system prophylaxis 4.