Burkitt lymphoma was first described by Dennis Burkitt in 1958 in the paper entitled ‘A sarcoma involving the jaws of African children’, following his observation of several children with multiple jaw tumours in Uganda, where he was working as a surgeon for the British government. These small round cell tumours were later recognised to be lymphomas. Burkitt defined a geographic ‘lymphoma belt’ with a high incidence of BL. The ‘lymphoma belt’ corresponds closely to the distribution of several insect-transmitted diseases leading to the hypothesis that BL might be driven by a virus. When MA Epstein, a pathologist with a special interest in virology, obtained tumour samples from Burkitt, he was able to demonstrate the presence of herpes-like particles in the tumours. The epidemiological and pathogenic relationship between the newly described Epstein-Barr virus (EBV) and African cases of BL was further demonstrated in several studies. In parallel, the observation that the ‘lymphoma belt’ mimicked the distribution of malaria and that successful malaria-eradication campaigns resulted in a decreased incidence of BL supported a role for malaria in the pathogenesis of BL, possibly by causing hyperplasia of B-lymphocytes and an increase in the circulation of EBV-infected memory B-cells. Whereas it is clear that both EBV and malaria play a significant role in the development of BL, the exact mechanism is still not well understood.
KeywordsPrimary Central Nervous System Lymphoma Central Nervous System Involvement Burkitt Lymphoma Immunocompetent Patient Central Nervous System Lymphoma
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