What is the treatment for Burkitt's lymphoma?

Treatment of Burkitt's Lymphoma

The treatment of Burkitt's lymphoma requires intensive chemotherapy regimens such as CODOX-M (cyclophosphamide, doxorubicin, vincristine with intrathecal methotrexate and cytarabine followed by high-dose systemic methotrexate) with rituximab, as standard CHOP regimens are inadequate for this aggressive lymphoma. 1

Diagnosis and Staging

Before initiating treatment, proper diagnosis and staging are essential:

• Essential diagnostic procedures:

  • Excisional or incisional biopsy (FNA or core needle biopsy alone is generally not suitable)
  • Hematopathology review with immunophenotyping
  • Cytogenetics or FISH to confirm t(8;14) or variants; MYC rearrangements
  • Typical immunophenotype: sIg+, CD10+, CD20+, TdT-, Ki67+ (100%), BCL2-, BCL6+

• Essential staging workup:

  • Physical exam with attention to node-bearing areas
  • CBC, differential, platelets
  • LDH (prognostic indicator - levels >1000 IU/L associated with worse prognosis)
  • Comprehensive metabolic panel and uric acid
  • Chest/abdominal/pelvic CT with contrast
  • Lumbar puncture (CNS involvement is common)
  • Bone marrow biopsy ± aspirate
  • HIV testing and Hepatitis B testing 1

Treatment Approach

First-line Treatment Regimens

1. Intensive chemotherapy regimens (in alphabetical order):

   • CALGB 9251 regimen: Cyclophosphamide and prednisone followed by cycles with ifosfamide/cyclophosphamide, high-dose methotrexate, leucovorin, vincristine, dexamethasone, and either doxorubicin, etoposide, or cytarabine; plus intrathecal therapy 1

   • CODOX-M ± rituximab: Original or modified protocol with cyclophosphamide, doxorubicin, vincristine with intrathecal methotrexate and cytarabine followed by high-dose systemic methotrexate (3 cycles) 1

   • Dose-adjusted EPOCH-R: Etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin plus rituximab 1, 2

   • LMB protocol: Recommended as an acceptable alternative treatment regimen, particularly in young adults 1

2. Addition of rituximab:

   • Rituximab should be added to intensive chemotherapy for all CD20+ Burkitt lymphoma
   • This addition significantly improves outcomes with 3-year event-free survival rates of 75% versus 62% without rituximab, and 3-year overall survival rates of 83% versus 70% 1

Important Treatment Considerations

• CHOP is not adequate therapy for Burkitt's lymphoma 1

• CNS prophylaxis: Intrathecal chemotherapy is essential due to high risk of CNS involvement 1

• Tumor lysis syndrome prevention: Due to rapid cell turnover, precautions must be taken to prevent tumor lysis syndrome with aggressive hydration and allopurinol or rasburicase

• Treatment should be administered at centers with expertise in managing this complex disease 1

• Special populations:

  • For elderly patients or those with comorbidities: Consider dose-adjusted regimens
  • For patients with renal impairment: EPOCH-R may be better tolerated 2
  • For HIV-positive patients: Combination with effective antiretroviral therapy is essential

Monitoring and Response Evaluation

• Regular monitoring of blood counts during treatment due to high risk of myelosuppression
• Febrile neutropenia is a common complication requiring prompt management
• Response evaluation should include repeat imaging of initially involved sites
• For patients with initial CNS or bone marrow involvement, repeat evaluation of these sites is necessary

Prognosis

Prognosis varies based on:

• Disease stage (Stage I-III has better outcomes than Stage IV)
• Initial LDH level (>1000 IU/L associated with worse prognosis)
• CNS involvement (poorer prognosis)
• Response to initial therapy

With modern intensive chemotherapy regimens plus rituximab, complete remission rates of 80-90% can be achieved, with long-term event-free survival rates of approximately 80% in patients with localized disease 1.

Common Pitfalls to Avoid

1. Using standard CHOP regimen: This is inadequate for Burkitt's lymphoma and will lead to treatment failure

2. Delaying treatment: Due to the rapid doubling time of Burkitt lymphoma cells, treatment should be initiated promptly after diagnosis

3. Inadequate CNS prophylaxis: Given the high risk of CNS involvement, intrathecal chemotherapy is essential

4. Underestimating tumor lysis risk: Aggressive preventive measures must be taken due to rapid tumor cell death during treatment

5. Inadequate supportive care: Intensive monitoring and management of complications are necessary during treatment

Burkitt's lymphoma is a highly aggressive but potentially curable malignancy when treated appropriately with intensive chemotherapy regimens including rituximab for CD20+ disease.