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Burkitt lymphoma
Background
Overview
Definition
Burkitt lymphoma is a highly aggressive B-cell non-Hodgkin's lymphoma characterized by rapid growth and a high proliferation rate. Three clinical variants share similar morphology, immunophenotype, and genetics, but they differ in etiology and epidemiology: endemic, sporadic, and HIV-related.
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Pathophysiology
The pathophysiology of Burkitt lymphoma involves a chromosomal translocation of the MYC gene. In most cases, the t(8;14) translocation places MYC under the control of immunoglobulin heavy chain enhancer elements, leading to its constitutive expression. Additional genetic alterations, such as mutations in CCND3, TP53, and CDKN2A, contribute to oncogenesis. In endemic regions, chronic EBV infection combined with exposure to P. falciparum is thought to increase genomic instability through activation-induced cytidine deaminase. Similarly, in HIV-associated cases, persistent B-cell stimulation and viral microRNAs further facilitate the oncogenic process.
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Epidemiology
The incidence of Burkitt lymphoma in the US is reported at 5.95 per 1,000,000 in men and 2.36 per 1,000,000 in women. Endemic Burkitt lymphoma accounts for up to 50% of childhood cancers in endemic regions, with an annual incidence of 40-50 per million children. Sporadic cases show peak incidence at around 11 years in children and at about 30 years in adults, representing less than 1-2% of adult lymphomas.
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Risk factors
Endemic variants are associated with EBV infection and primarily affect young boys in malaria-endemic regions. Sporadic forms are found in nonmalarial areas and may also be associated with EBV infection. The third variant is associated with HIV infection.
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Disease course
The sporadic variant commonly presents with abdominal involvement, characterized by abdominal pain, abdominal distension, gastrointestinal bleeding, nausea, and vomiting. It may also affect the head and neck region and, in rare cases, involve the CNS or mediastinum. The endemic variant most often presents with involvement of the jaw, facial, and periorbital regions. General systemic symptoms include fever, night sweats, weight loss, malaise, and lymphadenopathy.
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Prognosis and risk of recurrence
Intensive combination chemotherapy can achieve cure rates of up to 90%.
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Guidelines
Key sources
The following summarized guidelines for the evaluation and management of Burkitt's lymphoma are prepared by our editorial team based on guidelines from the European Reference Network on Rare Hematological Diseases (ERN-EuroBloodNet 2025) and the National Comprehensive Cancer Network (NCCN 2025).
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Screening and diagnosis
Classification and risk stratification
Diagnostic investigations
History and physical examination: as per NCCN 2025 guidelines, perform a physical examination with special attention to node-bearing areas, including Waldeyer's ring, liver size, and spleen size. Assess for B symptoms and performance status.
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Laboratory evaluation
Diagnostic imaging
Diagnostic procedures
Biopsy and histopathology: as per ERN-EuroBloodNet 2025 guidelines, perform a surgical biopsy or lymph node excision for histopathology examination, including attempting an imprint. Consider making a preliminary diagnosis rapidly and reliably using cytological analysis (bone marrow, serous effusion, or FNA biopsy of the lymph node) before the final and complete pathological analysis.
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Lumbar puncture
Bone marrow assessment
Medical management
First-line therapy: as per ERN-EuroBloodNet 2025 guidelines, offer immunochemotherapy in high-risk, fit patients with Burkitt lymphoma. Offer intensive (such as R-CODOX-M / R-IVAC, LMB-R, GMALL-B-ALL/NHL 2002) or intermediate-intensive (such as DA-EPOCH-R) regimens, as they report higher overall survival rates compared to lower dose regimens (such as R-CHOP).
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Second-line therapy
Prevention of tumor lysis syndrome