Burkitt lymphoma- a rare but challenging lymphoma

Best Pract Res Clin Haematol. 2018 Sep;31(3):279-284. doi: 10.1016/j.beha.2018.07.013. Epub 2018 Jul 23.

Abstract

Burkitt lymphoma (BL) is a rare, aggressive subtype of non-Hodgkin lymphoma affecting approximately 1500 patients per year. Three forms of BL exist (sporadic, endemic, immunodeficiency associated) and the endemic form was first discovered as being driven by the Epstein Barr virus in areas of the world where malaria is prevalent. BL has the characteristic t8; 14 cytogenetic translocation that leads to constitutive activation of the MYC gene, which drives BL cell division. Therapy of BL has resulted in cure for many patients but significant toxicity and treatment related complications remains problematic in the approach to BL therapy. Treatment options for relapsed and refractory disease remain limited however novel treatments are being studied to block MYC activation, and cold lead to promising options for patients with relapsed and refractory disease.

Keywords: 14 translocation; Burkitt lymphoma; Cytotoxic chemotherapy; Epstein barr virus; MYC gene; t8.

Publication types

  • Review

MeSH terms

  • Burkitt Lymphoma* / genetics
  • Burkitt Lymphoma* / metabolism
  • Burkitt Lymphoma* / therapy
  • Burkitt Lymphoma* / virology
  • Chromosomes, Human, Pair 14 / genetics*
  • Chromosomes, Human, Pair 8 / genetics
  • Epstein-Barr Virus Infections* / genetics
  • Epstein-Barr Virus Infections* / metabolism
  • Epstein-Barr Virus Infections* / therapy
  • Herpesvirus 4, Human* / genetics
  • Herpesvirus 4, Human* / metabolism
  • Humans
  • Proto-Oncogene Proteins c-myc / genetics
  • Proto-Oncogene Proteins c-myc / metabolism
  • Translocation, Genetic

Substances

  • MYC protein, human
  • Proto-Oncogene Proteins c-myc