Burkitt lymphoma

Definition

Burkitt lymphoma (BL) is a highly aggressive B-cell non-Hodgkin lymphoma characterized by rapid proliferation and translocations involving the MYC gene (usually t(8;14)(q24;q32)). It is considered a hematologic emergency because of its fast growth and high risk of tumor lysis syndrome (TLS).

Epidemiology

• Most common in children and young adults.
• Three clinical variants:
  1. Endemic – Africa, associated with EBV, often jaw/facial bone involvement.
  2. Sporadic – Worldwide, often abdominal involvement.
  3. Immunodeficiency-related – HIV-associated.

Pathophysiology / Molecular Markers

• MYC gene translocation → uncontrolled cell proliferation.
• Highly proliferative (Ki-67 nearly 100%).
• EBV positivity in endemic variant.

Clinical Presentation

• Rapidly enlarging mass (jaw, abdomen, or lymph nodes)
• B-symptoms: fever, night sweats, weight loss
• Possible CNS involvement in some cases

Standard Treatment

BL is highly chemosensitive; intensive short-course chemotherapy is standard:

• CODOX-M/IVAC regimen:
  • Cyclophosphamide, Vincristine, Doxorubicin, High-dose Methotrexate / Ifosfamide, Etoposide, Cytarabine
• DA-EPOCH-R regimen (dose-adjusted, includes Rituximab)
• CNS prophylaxis with intrathecal methotrexate or cytarabine

Pharmacist-Relevant Considerations

• Tumor lysis syndrome (TLS):
  • High risk due to rapid proliferation; allopurinol or rasburicase for prophylaxis.
  • Aggressive hydration and electrolyte monitoring required.
• Chemotherapy toxicities:
  • Myelosuppression → infection risk
  • Mucositis (especially with methotrexate)
  • Cardiotoxicity (doxorubicin)
  • Neurotoxicity (vincristine)
• Drug interactions:
  • Methotrexate and leucovorin rescue require careful timing; watch nephrotoxic or nephroactive drugs.
• Supportive care:
  • Antiemetics, growth factors (G-CSF), infection prophylaxis as indicated.
• Rituximab:
  • Infusion reactions, hepatitis B reactivation risk; pre-screening required.