Description

• Burkitt lymphoma is a mature B-cell non-Hodgkin lymphoma (NHL) 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
  • Sporadic
  • 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), thus treated similarly
• 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

Pediatric Considerations
Common age group (>30% of all childhood NHL cases in the United States)

Geriatric Considerations
Unusual in this age group. Toxicity with chemotherapy may be increased in the elderly.

Pregnancy Considerations
With aggressive treatment, good maternal and fetal outcome

Epidemiology

• Varies by disease form
• Endemic
  • One of most common tumors of childhood in Africa; most frequently occurring in children age 4 to 7 years
  • Rare in adults
• Sporadic
• 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)

Incidence
Rare in the United States, incidence 0.27/100,000 person-years; 50 times more common in endemic regions of Africa

Prevalence
Composes <1% of adult 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

Risk Factors

Pediatric Considerations
Endemic: Children with early acquisition of EBV infection are at increased risk. Coinfection with malaria and EBV increases incidence 100-fold.

General Prevention

No known methods to prevent Burkitt lymphoma

Commonly Associated Conditions

• EBV infection
• Immunodeficiency, especially AIDS