Childhood lymphoma resembling “burkitt tumor” in the United States

O’Conor, Gregory T.; Rappaport, Henry; Smith, Edward B.

doi:10.1002/1097-0142(196504)18:4<411::aid-cncr2820180403>3.0.co;2-5

Cited by 178 publications

(22 citation statements)

References 11 publications

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“…In fact, the unusually high frequency of jaw tumours occurring mainly in young children, led many to think of it as a separate disease and referred to it as the 'African lymphoma.' In the mid-1960s several pathologists described lymphomas in Europe and the USA that were indistinguishable at a histological level, and often also clinically, from African BL (doubtless because the presence of jaw tumours drew attention to them), and it became clear that this was not a uniquely African disease, although it was much more common in Africa (Dorfman, 1965;O'Conor et al, 1965;Wright, 1966).…”

Section: The Discovery Of Burkitt Lymphoma In Africamentioning

confidence: 99%

Epidemiology: clues to the pathogenesis of Burkitt lymphoma

2012

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SummaryThe two major epidemiological clues to the pathogenesis of Burkitt lymphoma (BL) are the geographical association with malaria -BL incidence relates to the malaria transmission rate -and early infection by Epstein-Barr virus (EBV). Both agents cause B cell hyperplasia, which is almost certainly an essential component of lymphomagenesis in BL. The critical event in lymphomagenesis is the creation of a MYC translocation, bringing the MYC gene into juxtaposition with immunoglobulin genes and causing its ectopic expression, thereby driving the proliferation of BL cells. It is highly likely that such translocations are mediated by the activation-induced cytidine deaminase (AID) gene, which is responsible for hypervariable region mutations as well as class switching. Stimulation of the Toll-like receptor 9 by malaria-associated agonists induces AID, providing a mechanism whereby malaria could directly influence BL pathogenesis. EBV-containing cells must reach the memory cell compartment in order to survive throughout the life of the individual, which probably requires traversal of the germinal centre. Normally, cells that do not produce high affinity antibodies do not survive this passage, and are induced to undergo apoptosis. EBV, however, prevents this, and in doing so may also enhance the likelihood of survival of rare translocation-containing cells.

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Section: The Discovery Of Burkitt Lymphoma In Africamentioning

confidence: 99%

Epidemiology: clues to the pathogenesis of Burkitt lymphoma

2012

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“…The question whether this lymphoma is unique and restricted to endemic regions of the world, or whether it can also be found elsewhere, arose with 3 reports in -1966(Dorfman, 1965O'Conor et al, 1965;Wright, 1966). The answer should have been provided by the plethora of reports from many countries, published between 1966 and 1970 (see: Philip et al, 1980b;Lenoir et al, unpublished).…”

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confidence: 99%

Burkitt-type lymphoma in France among non-Hodgkin malignant lymphomas in Caucasian children

Philip¹,

Lenoir²,

Bryon³

et al. 1982

Br J Cancer

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In a retrospective analysis of 87 cases of Caucasian childhood non-Hodgkin malignant lymphoma (NHML) from Lyon, France, all the case were diffuse lymphomas, but 47 were diagnosed as monomorphic small non-cleaved NHML, pathologically indistinguishable from Burkitt's lymphoma (BL). BL could then be the most frequent childhood lymphoma in France. This homogeneous series allows better definition of the characteristics of BL within NHML. Age distribution is similar to that of endemic BL, with a sex ratio of 3.7/1. Abdominal masses are initially present in 68% of the cases, whereas jaw is involved in only 4%. The disease is characterized by its overwhelming evolution in the absence of therapy. However, complete remission (CR) is usually obtained after the first chemtherapy regimen. Most relapses occur at 3-8 months. Death could be related to cerebrospinal fluid (CSF) involvement, local recurrence or secondary marrow involvement. Ninety per cent of the patients alive with no evidence of disease (NED) 8 months after CR can be considered as definitely cured. Our study on Caucasian children with NHML indicates that, from histological and clinical criteria, nearly half the cases are very similar to African BL. Even though EBV rarely associated with our cases, BL could be a worldwide lymphoma.

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“…It is also known that there is a high incidence in New Guinea, which has a climate similar to that of tropical Africa (ten Seldam et al, 1966). However, cases have been identified in the U.S.A. and England (O'Conor et al, 1965 ;Dorfman, 1965;Wright, 1965). These areas are well outside the originally defined parameters, and, in the case of England, completely outside the known areas of distribution of any arthropod-borne virus.…”

Section: Discussionmentioning

confidence: 99%