Burkitt lymphoma
Burkitt lymphoma is an aggressive mature B-cell cancer marked by rapid tumor growth and usually by a rearrangement that activates the MYC gene.
MYC: A gene encoding a transcription factor that regulates cell growth, metabolism, and proliferation. Burkitt lymphoma most often develops when a rearrangement places MYC under unusually active regulatory control.
Endemic Burkitt lymphoma: A Burkitt lymphoma variant prevalent in equatorial Africa and Papua New Guinea, often involving the jaw and associated with Epstein–Barr virus. It is the classic geographic form and commonly presents with facial bone tumors in children.
B cell: A lymphocyte that produces antibodies and participates in adaptive immunity. Burkitt lymphoma arises from mature B cells, usually with a germinal-center phenotype.
R-CHOP: A chemoimmunotherapy regimen combining rituximab with cyclophosphamide, doxorubicin, vincristine, and prednisone. This common regimen for other aggressive B-cell lymphomas is generally inadequate for Burkitt lymphoma.
Denis Parsons Burkitt: An Irish surgeon who described the distinctive childhood jaw tumors later associated with Burkitt lymphoma. His observations in equatorial Africa led to recognition of the endemic form.
Immunoglobulin gene rearrangement: The DNA recombination process that assembles antibody genes in developing B cells. MYC is commonly moved next to an immunoglobulin gene enhancer in this lymphoma.
Sporadic Burkitt lymphoma: A Burkitt lymphoma variant occurring worldwide, often presenting with abdominal tumors. Its abdominal presentation is a frequent contrast with the jaw tumors of endemic disease.
Germinal center: A transient structure in lymph nodes where activated B cells mutate antibody genes and undergo selection. The tumor’s molecular and immunophenotypic features point to a germinal-center B-cell origin.
CODOX-M/IVAC: An intensive chemotherapy program using alternating CODOX-M and IVAC courses. It is one established regimen designed for the disease’s fast growth and risk of central nervous system spread.
Uganda Cancer Institute: A cancer research and treatment center in Kampala, Uganda, established in 1967. It became a center for studying the lymphoma first characterized through African clinical observations.