What is the WHO classification of Burkitt lymphoma/Burkitt-like lymphoma (BL/BLL)?

Updated: Dec 20, 2019
  • Author: Ali H Kanbar, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

The 2008 WHO modification of the REAL classification of NHL is based on morphology and cell lineage. The WHO classification identifies the following three clinical variants of Burkitt lymphoma (BL) [25] :

  • Endemic (eBL) – The most common form of childhood malignancy in equatorial Africa, associated with Epstein-Barr virus (EBV) infection

  • Sporadic (sBL) – The majority of cases are in the United States and Europe; up to 30% are associated with EBV

  • Immunodeficiency associated – Occurs in patients with HIV infection, post-transplantation immunosuppression, and congenital immunodeficiency

The 2016 revision of the WHO classification recognizes the following two molecular entities [2] :

  • Burkitt lymphoma - TCF3 or ID3 mutations in up to about 70% of cases
  • Burkitt-like lymphoma with 11q aberration - New provisional entity that closely resembles Burkitt lymphoma but lacks MYC rearrangement and has some other distinctive features

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