Burkitt lymphoma (BL) is an aggressive, very fast-growing B-cell non-Hodgkin lymphoma characterized by MYC oncogene translocation and near 100% Ki-67 proliferation index.
Clinical Subtypes (WHO Grouping)
• Endemic (African) BL: Predominantly in equatorial Africa and Papua New Guinea among children (peak age 4-7). Presents as jaw/facial bone tumor; strongly linked to EBV and holoendemic malaria.
• Sporadic BL: Worldwide (North America & Europe). Affects children and young adults; presents as abdominal masses (ileocecal, mesentery, ovaries, kidneys).
• Immunodeficiency-Associated BL: Occurs in patients with HIV infection or post-transplant immunosuppression.
Ann Arbor Staging & Diagnostic Pathology
Tissue diagnosis reveals classic 'starry-sky' microscopic pattern. Cytogenetics showing MYC translocation confirms diagnosis.
Ann Arbor Staging (Stages I-IV): Evaluates single node regions up to Stage IV marrow or CNS involvement. Diagnostic lumbar puncture is required.
Intensive Chemotherapy & TLS Prevention
Curative therapy relies on short-course multi-agent chemotherapy (cyclophosphamide, vincristine, doxorubicin, methotrexate, cytarabine) + Rituximab (anti-CD20).
CNS intrathecal chemo prophylaxis is mandatory. Tumor Lysis Syndrome (TLS) prophylaxis with hydration and rasburicase/allopurinol is imperative.