KEGG   DISEASE: H00008Help
Entry
H00008                      Disease                                

Name
Burkitt lymphoma
Description
Burkitt lymphoma (BL) is a highly aggressive mature B-cell non-Hodgkin's lymphoma consisting of endemic, sporadic, and immunodeficiency-associated variants. Endemic BL (eBL) affects children and young adults in Africa and some other geographical areas and carries Epstein-Barr virus (EBV) in more than 95% of cases. In contrast, sporadic BL (sBL) among adolescents in Europe and North America are mostly EBV-negative. A third type of BL is associated with HIV-infection in adults. All of these subtypes possess chromosomal rearrangements of the c-myc oncogene, the genetic hallmark of BL that contributes to lymphomagenesis through alterations in cell cycle regulation, cellular differentiation, apoptosis, cellular adhesion, and metabolism. Many BL carry point mutation in the p53 tumor suppressor gene or other defects in the p14ARF-MDM2-p53 pathway, and inactivation of the p16INK4a gene by promoter methylation or homozygous deletion. This indicates that disruption of both the pRb and p53 tumor suppressor pathways is critical for BL development.
Category
Cancer
BRITE hierarchy
Pathway
Viral carcinogenesis
Gene
MYC-IgH (translocation) [HSA:4609] [KO:K04377]
p53 (mutation) [HSA:7157] [KO:K04451]
p16/INK4A (promoter methylation) [HSA:1029] [KO:K06621]
Carcinogen
*Azathioprine [DG:DG00743]
*Ethylene oxide [CPD:C06548]
*2,3,7,8-Tetrachlorodibenzo-para-dioxin [CPD:C07557]
*: Although this is a risk factor for non-Hodgkin lymphoma in general, the strength of association with different histological tumor types is not well understood.
Pathogen
EBV (Epstein-Barr Virus)
*Human immunodeficiency virus type 1 (infection with) [VGNM:NC_001802]
*: Although this is a risk factor for non-Hodgkin lymphoma in general, the strength of association with different histological tumor types is not well understood.
Marker
c-MYC [HSA:4609]
Drug
Cyclophosphamide [DG:DG00675]
Vincristine [DG:DG00690]
Doxorubicin [DG:DG00696]
Prednisone (CHOP) [DG:DG00094]
Rituximab [DR:D02994]
Comment
ICD-O: 9687/3, Tumor type: Burkitt lymphoma
Other DBs
ICD-10: 
MeSH: 
Reference
PMID:12191637 (gene, carcinogen, tumor type)
  Authors
Lindstrom MS, Wiman KG.
  Title
Role of genetic and epigenetic changes in Burkitt lymphoma.
  Journal
Semin Cancer Biol 12:381-7 (2002)
Reference
PMID:15803153 (gene, carcinogen)
  Authors
Kuppers R.
  Title
Mechanisms of B-cell lymphoma pathogenesis.
  Journal
Nat Rev Cancer 5:251-62 (2005)
Reference
PMID:16062089 (carcinogen)
  Authors
Patel P, Moazzam N, Potti A, Mehdi SA.
  Title
Azathioprine induced hodgkin lymphoma: a case report and review of literature.
  Journal
Am J Clin Oncol 28:427-8 (2005)
Reference
PMID:15760280 (carcinogen)
  Authors
Schottenfeld D, Beebe-Dimmer JL.
  Title
Advances in cancer epidemiology: understanding causal mechanisms and the evidence for implementing interventions.
  Journal
Annu Rev Public Health 26:37-60 (2005)
Reference
PMID:15660110 (carcinogen)
  Authors
Luch A.
  Title
Nature and nurture - lessons from chemical carcinogenesis.
  Journal
Nat Rev Cancer 5:113-25 (2005)
Reference
PMID:12464351 (carcinogen)
  Authors
Kolman A, Chovanec M, Osterman-Golkar S.
  Title
Genotoxic effects of ethylene oxide, propylene oxide and epichlorohydrin in humans: update review (1990-2001).
  Journal
Mutat Res 512:173-94 (2002)
Reference
PMID:17530489 (carcinogen)
  Authors
Abnet CC.
  Title
Carcinogenic food contaminants.
  Journal
Cancer Invest 25:189-96 (2007)
Reference
PMID:16848772 (marker)
  Authors
Cogliatti SB, Novak U, Henz S, Schmid U, Moller P, Barth TF.
  Title
Diagnosis of Burkitt lymphoma in due time: a practical approach.
  Journal
Br J Haematol 134:294-301 (2006)
Reference
PMID:15023043 (marker)
  Authors
Frost M, Newell J, Lones MA, Tripp SR, Cairo MS, Perkins SL.
  Title
Comparative immunohistochemical analysis of pediatric Burkitt lymphoma and diffuse large B-cell lymphoma.
  Journal
Am J Clin Pathol 121:384-92 (2004)
Reference
(drug)
  Authors
McPhee SJ, Papadakis MA, Tierney LM (ed).
  Title
Current Medical Diagnosis & Treatment 2007, Forty-Sixth Edition
  Journal
The McGraw-Hill Companies, Inc. (2007)

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