How is Burkitt lymphoma/Burkitt-like lymphoma (BL/BLL) classified?

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

Historically, Burkitt lymphoma was termed malignant small noncleaved lymphoma in patients presenting with a solid tumor or nodal mass, whereas patients who had greater than 25% bone marrow involvement were considered to have French American British (FAB) L3 type acute lymphoblastic leukemia (L3 ALL). The 2016 World Health Organization (WHO) classification of lymphoid neoplasms identifies Burkitt lymphoma, in which TCF3 or ID3 mutations are present in up to about 70% of cases, and Burkitt-like lymphoma with 11q aberration, a new provisional entity that closely resembles Burkitt lymphoma but lacks MYC rearrangement and has some other distinctive features. [2]

The dysregulation and mutation of the c-myc oncogene that characterizes Burkitt lymphoma often results from a translocation between chromosomes 8 and 14, t(8;14)(q24;q32). Other translocations are also reported to cause c-myc overexpression, including t(2;8)(p12;q24) and t(8;22)(q24;q11). However, according to the WHO classification of lymphoid neoplasms, diagnosis of Burkitt lymphoma can still be made even in the absence of c-myc rearrangement, if other clinical, morphologic, and immunophenotypic findings support that diagnosis. [2]

Three distinct forms of Burkitt lymphoma are identified: (1) endemic (African), (2) sporadic, and (3) immunodeficiency-associated subtypes. Although these forms differ in their clinical presentation and their epidemiology, they share the same aggressive clinical behavior and are histologically identical.

The sporadic variant (sBL) is present in North America and Western Europe, and the endemic variant (eBL) is observed in equatorial Africa. Immunodeficiency-associated Burkitt lymphoma occurs most commonly in patients with human immunodeficiency virus (HIV) infection, but it has also been reported in the posttransplantation setting [3] as well as in congenital immunodeficiency patients. [4] Immunodeficiency-associated Burkitt lymphoma accounts for about 30% of lymphomas in HIV patients. [5]

Burkitt lymphoma was originally described in children, but this disease is also observed in adult patients. Burkitt lymphoma is one of the fastest growing malignancies in humans, with a growth fraction close to 100% and a doubling time of around 25 hours. [6]

Burkitt and Burkitt-like lymphomas have a rapid and aggressive clinical course, commonly presenting in children and young adults, with frequent bone marrow and central nervous system (CNS) involvement. These are considered to be medical emergencies and require immediate diagnostic and therapeutic intervention.


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