ABSTRACT
Introduction 538 Malaria and African Burkitt lymphoma 539 Epstein-Barr virus association with Burkitt lymphoma 540 Epstein-Barr virus latent and lytic virus cycles in normal 541
and neoplastic cells Avoiding immune surveillance 542 Pathogenetic contribution of Epstein-Barr virus 543 Epstein-Barr virus strain differences 543 Molecular abnormalities in Burkitt lymphoma 544 Translocations in relationship to B cell differentiation 544 Molecular differences in Burkitt lymphoma from different 545
geographic regions
Consequences of the MYC-Ig translocations 545 Antagonism between MYC and the Epstein-Barr virus 547
growth program Immunoglobulin hypervariable region mutations and 548
Burkitt lymphoma subtype Mechanism of activation of MYC 548 Other molecular abnormalities 549 Alternative molecular mechanisms of lymphomagenesis 549 The definition of Burkitt lymphoma 549 A molecular definition 550 Key points 552 References 553
Burkitt lymphoma (BL) was first recognized as a distinct clinicopathologic entity in Africa. The eponymous designation is probably well deserved, since Denis Burkitt, a surgeon working in Kampala, Uganda, can literally be said to have put the tumor ‘on the map’ even though others, primarily pathologists,1-5 had recognized the high frequency of both jaw tumors and lymphomas in African children prior to the publication of Burkitt’s classical paper in 1958.6
Burkitt reported 38 Ugandan children with an unusual ‘sarcoma’ of the jaw that was by far the most frequent childhood cancer in the Kampala Cancer Registry. He described the characteristic clinical features of the jaw tumors and the unusual pattern of associated sites of involvement (in part based on autopsies) that included intraabdominal organs, and salivary and endocrine glands, but rarely the spleen or lymph nodes. At about the same time, O’Conor and Davis, pathologists also working at Mulago Hospital, Kampala, who had been conducting a survey of the malignant tumors of children diagnosed in their department (the major reference center for Uganda), confirmed Davis’s much earlier observation2 that approximately half of all the childhood cancers in the Mulago series were tumors of the reticuloendothelial system.7 O’Conor
the cases described by Burkitt, which he recognized as lymphomas.8
