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-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Mature B-cell neoplasms
    2A85  Other specified mature B-cell neoplasms or 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 p53-mediated transcription
N00224  EBV EBNALP RBP-Jk-mediated transcription
N00225  EBV EBNA2 to RBP-Jk-mediated transcription
N00226  EBV EBNA3A/3B/3C to RBP-Jk-mediated transcription
N00262  EBV EBNA3C to intrinsic apoptotic pathway
N00263  EBV EBNA3C to p53-mediated transcription
N00264  EBV EBNA3C to p27-Cell cycle G1/S
N00265  EBV LMP1 to NFKB signaling pathway
N00266  EBV LMP2A to PI3K signaling pathway
N00466  EBV BPLF1 to TLR2/4-NFKB signaling pathway
N00467  EBV BPLF1 to TLR2/4-NFKB signaling pathway
N00468  EBV BPLF1 to TLR2/4-NFKB signaling pathway
N00470  EBV BGLF4 to RIG-I-like receptor signaling pathway
N00471  EBV LMP2A/2B to IFN signaling pathway
N00472  EBV LMP1 to IFN signaling pathway
N00473  EBV BGLF4 to IFN signaling pathway
N00474  EBV BHRF1 to intrinsic apoptotic pathway
N00475  EBV BHRF1 to intrinsic apoptotic pathway
N00476  EBV BHRF1 to intrinsic apoptotic pathway
N00477  EBV BHRF1 to crosstalk between extrinsic and intrinsic apoptotic pathways
N00478  EBV BARF1 to intrinsic apoptotic pathway
N00479  EBV BNLF2a to antigen processing and presentation by MHC class I molecules
N00480  EBV BILF1 to antigen processing and presentation by MHC class I molecules
N00481  EBV BZLF1 to p53-mediated transcription
N00482  EBV EBNA3C to p27-Cell cycle G1/S
N00483  EBV EBNA3C to cell cycle G1/S
N00484  EBV EBNA3C to cell cycle G1/S
N00485  EBV LMP1 to PI3K signaling pathway
N00486  EBV LMP1 to Jak-STAT signaling pathway
N00488  EBV LMP2A to BCR signaling pathway
 » show all
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-11: 2A85.6
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)
DOI:10.1097/01.coc.0000168670.28659.8d
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

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