KEGG   DISEASE: Burkitt lymphomaHelp
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
Human diseases [BR:br08402]
 Cancers
  Cancers of haematopoietic and lymphoid tissues
   H00008  Burkitt lymphoma
Human diseases in ICD-10 classification [BR:br08403]
 2. Neoplasms (C00-D48)
  C81-C96  Malignant neoplasms, stated or presumed to be primary, of lymphoid, haematopoietic and related tissue
   C83  Diffuse non-Hodgkin's lymphoma
    H00008  Burkitt lymphoma
Tumor markers [br08442.html]
 H00008
BRITE hierarchy
Pathway
hsa05203  Viral carcinogenesis
hsa05169  Epstein-Barr virus infection
Network
N00223  EBV EBNA1 to host-cell-protein inhibition
N00224  EBV EBNALP binding to host-cell-protein
N00225  EBV EBNA2 binding to host-cell-protein
N00226  EBV EBNA3A/3B to host-cell-protein inhibition
N00262  EBV EBNA3C to host-cell-protein inhibition
N00263  EBV EBNA3C to host-cell-protein activation
N00264  EBV EBNA3C binding to host-cell-protein
N00265  EBV LMP1 to host-cell-protein activation
N00266  EBV LMP2 to host-cell-protein inhibition
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]
Pathogen
Human herpesvirus 4 (Epstein-Barr virus) [GN:T40079]
Human immunodeficiency virus 1 (HIV-1) [GN:T40001]
Drug
Cyclophosphamide [DR:D00287]
Carmustine [DR:D00254]
Doxorubicin hydrochloride [DR:D01275]
Rituximab [DR:D02994] (CD20-positive)
Plerixafor [DR:D08971]
Comment
ICD-O: 9687/3, Tumor type: Burkitt lymphoma
Although the carcinogens listed and infection with HIV-1 are risk factors for non-Hodgkin lymphoma in general, the strength of association with different histological tumor types is not well understood.
Other DBs
ICD-10: C83.7
MeSH: D002051
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)
DOI:10.1016/S1044-579X(02)00058-5
Reference
PMID:15803153 (gene, carcinogen)
  Authors
Kuppers R.
  Title
Mechanisms of B-cell lymphoma pathogenesis.
  Journal
Nat Rev Cancer 5:251-62 (2005)
DOI:10.1038/nrc1589
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)
DOI:10.1146/annurev.publhealth.26.021304.144402
Reference
PMID:15660110 (carcinogen)
  Authors
Luch A.
  Title
Nature and nurture - lessons from chemical carcinogenesis.
  Journal
Nat Rev Cancer 5:113-25 (2005)
DOI:10.1038/nrc1546
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)
DOI:10.1016/S1383-5742(02)00067-4
Reference
PMID:17530489 (carcinogen)
  Authors
Abnet CC.
  Title
Carcinogenic food contaminants.
  Journal
Cancer Invest 25:189-96 (2007)
DOI:10.1080/07357900701208733
Reference
(drug)
  Authors
Papadakis MA and McPhee SJ |(ed)
  Title
Current Medical Diagnosis & Treatment 2015, Fifty-Fourth Edition
  Journal
McGraw Hill Education

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