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Lymphoma, Burkitt

Lymphoma, Burkitt is a topic covered in the 5-Minute Clinical Consult.

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Basics

Description

  • 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
    • 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)
  • 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 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 per 100,000 person-years; 50 times more common in endemic regions of Africa

Prevalence
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

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

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Citation

Stephens, Mark B., et al., editors. "Lymphoma, Burkitt." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116361/all/Lymphoma__Burkitt.
Lymphoma, Burkitt. In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116361/all/Lymphoma__Burkitt. Accessed April 20, 2019.
Lymphoma, Burkitt. (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116361/all/Lymphoma__Burkitt
Lymphoma, Burkitt [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 April 20]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116361/all/Lymphoma__Burkitt.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Lymphoma, Burkitt ID - 116361 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116361/all/Lymphoma__Burkitt PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -