What is the diagnosis and treatment for Burkitt lymphoma/leukemia (B cell non-Hodgkin lymphoma)?

From the Guidelines

The addition of rituximab to intensive chemotherapy is strongly recommended for the treatment of mature B cell leukemia Burkitt type, as it has been shown to improve outcomes in adult patients. This recommendation is based on the results of several studies, including a large multicenter trial by the German Multicenter Study Group for Adult Acute Lymphoblastic Leukemia (GMALL) 1, which demonstrated a high complete remission (CR) rate of 86% with rituximab-eChT. The Group for Research on Adult ALL (GRAALL) and the Lymphoma Study Association (LYSA) study group also evaluated rituximab-eChT in a randomized trial in Burkitt leukemia/lymphoma and found significant improvements in 3-year event-free survival (EFS) rate and 3-year overall survival (OS) rate 1.

The treatment of mature B cell leukemia Burkitt type typically involves combination chemotherapy regimens such as CODOX-M/IVAC (cyclophosphamide, vincristine, doxorubicin, high-dose methotrexate alternating with ifosfamide, etoposide, and high-dose cytarabine) or DA-EPOCH-R (dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab). For adults, a typical regimen might include 3-4 cycles of DA-EPOCH-R with CNS prophylaxis using intrathecal methotrexate. Key points to consider in the treatment of mature B cell leukemia Burkitt type include:

• The importance of prompt initiation of treatment at specialized centers experienced in managing aggressive lymphomas due to the rapid doubling time of this malignancy
• The need for supportive care, including tumor lysis syndrome prevention with allopurinol or rasburicase, hydration, and electrolyte monitoring
• The importance of close monitoring with frequent blood counts, chemistry panels, and imaging studies to assess response
• The consideration of rituximab in combination with intensive chemotherapy as the standard of care for adult Burkitt lymphoma/leukaemia, as recommended by the ESMO clinical practice guideline interim update on the use of targeted therapy in acute lymphoblastic leukaemia 1.

Overall, the treatment of mature B cell leukemia Burkitt type requires a comprehensive approach that incorporates intensive chemotherapy, supportive care, and close monitoring, with the addition of rituximab as a key component of the treatment regimen.

From the FDA Drug Label

RITUXAN is indicated for the treatment of pediatric patients aged 6 months and older with: Previously untreated, advanced stage, CD20-positive diffuse large B-cell lymphoma (DLBCL), Burkitt lymphoma (BL), Burkitt-like lymphoma (BLL) or mature B-cell acute leukemia (B-AL) in combination with chemotherapy.

The rituximab is indicated for the treatment of mature B-cell leukemia Burkitt type in pediatric patients aged 6 months and older in combination with chemotherapy 2, 2, 2.

• The dosage for pediatric patients is 375 mg/m2 2.
• Rituximab should be administered only as an intravenous infusion 2, 2.
• Premedication is recommended before each infusion 2, 2.

From the Research

Characteristics of Mature B Cell Leukemia Burkitt Type

• Burkitt lymphoma (BL) is a mature B-cell non-Hodgkin lymphoma with an aggressive clinical course 3
• BL is characterized by marked tumor proliferation resulting from translocation of the MYC oncogene 4
• The disease is highly aggressive and can quickly disseminate to extranodal sites, including the bone marrow and central nervous system (CNS) 4

Treatment and Prognosis

• BL is highly sensitive to chemotherapy, and patients who tolerate highly intensive combination chemotherapy regimens are frequently cured 4
• The addition of rituximab to hyper-CVAD may improve outcome in adult BL or B-ALL, particularly in elderly patients 5
• 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 4
• The hyper-cyclophosphamide, vincristine, Adriamycin, and dexamethasone plus rituximab (CVAD-R) regimen includes systemic and intrathecal CNS-directed therapy to treat and prevent CNS disease, and has been shown to be effective in preventing CNS relapse 6

Clinical Variants and Risk Factors

• Distinct clinical variants of BL include endemic, sporadic, and immunodeficiency-associated cases 4, 7
• Patients with low-risk BL can be cured with just 3 cycles of DA-EPOCH-RR, while patients with CNS involvement remain at high risk for early death 4
• Age and CNS involvement are independent predictors for overall survival (OS) and relapse-free survival (RFS) 6
• Baseline bone marrow (BM) and CNS involvement are associated with a higher cumulative incidence of relapse (CIR) 6