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- Burkitt lymphoma is a mature B-cell neoplasm arising from lymph node germinal centers.
- Highly aggressive, rapidly growing malignancy
- Can present as lymphoma or leukemia
- Three distinct forms differing in epidemiology, clinical presentation, and genetics
- Endemic or African
- Immunodeficiency related
- HIV/AIDS related
- Postsolid organ transplant
- Congenital immunodeficiency
- Associated with Epstein-Barr virus (EBV)
- Almost 100% of endemic cases
- Up to 30% of sporadic cases
- Characteristic chromosome translocation (t[8;14])
- Similar disease characteristics to diffuse large B-cell lymphoma (DLBCL)
- System(s) affected: hematologic, lymphatic, CNS
- Synonym(s): mature B-cell high-grade lymphoma; mature B-cell acute lymphoblastic leukemia; L3 type (French-American-British [FAB] classification); Burkitt cell leukemia
Common age group (30% of cases in the United States)
Unusual in this age group. Toxicity with chemotherapy may be increased in the elderly.
With aggressive treatment, good maternal and fetal outcome
- Varies by disease form
- One of most common tumors of childhood in Africa; most frequently occurring in children age 4 to 7 years
- Rare in adults
- In the United States, trimodal peaks of age incidence around ages 10, 40, and 75 years
- More common in Caucasians
- Predominant sex: male > female (3:1 or 4:1)
Rare in the United States, incidence 0.27 per 100,000 person-years; 50 times more common in endemic regions of Africa
Composes <1% of adult non-Hodgkin lymphoma (NHL); accounts for 30–40% of NHL in children in the United States and Western Europe
Etiology and Pathophysiology
- Activation and overexpression of c-myc oncogene (1)
- Monoclonal proliferation of B lymphocytes resulting from dysregulation of c-myc
- Translocation of c-myc to immunoglobulin coding regions results in constitutive expression of gene product.
- EBV-infected cells in germinal center reactions may increase the risk of translocation.
- Poorly regulated proliferation of genetically unstable B cells increases chance of translocations:
- Immunodeficient patients with persistent generalized lymphadenopathy and polyclonal B-cell activation
Endemic: Children with early acquisition of EBV infection are at increased risk. Coinfection with malaria and EBV increases incidence 100-fold.
No known methods to prevent Burkitt lymphoma
Commonly Associated Conditions
- EBV infection
- Immunodeficiency, especially AIDS