KEGG   DISEASE: H00007Help
Entry
H00007                      Disease                                

Name
Hodgkin lymphoma
Description
Hodgkin's lymphoma (HL) is one of the most frequent lymphomas in the Western world and often affects young adults. HL is subdivided into classical and nodular lymphocyte-predominant forms. About 95% of cases are classical HL, and 5% are nodular lymphocyte-predominant HL (NLPHL). A characteristic feature of HL is the rareness of the tumor cells, which are called Hodgkin's and Reed/Sternberg (HRS) cells in classical HL and lymphocytic and histiocytic (L&H) cells in NLPHL. These cells represent only about 1% of the cellular infiltrate, while the vast majority of infiltrating cells are T lymphocytes, histiocytes, eosinophilic granulocytes and plasma cells. HRS cells show constitutive activity of both the classical and alternative NF-{kappa}B signalling pathways, which is probably a major pathogenetic mechanism in Hodgkin's lymphoma. The NF-{kappa}B activity in HRS cells is probably mediated by diverse mechanisms: receptor signalling through CD40, RANK, BCMA, and TACI, genomic REL amplification, destructive mutations in IKBA and IKBE. In HL pathogenesis associated with Epstein-Barr virus infection, the activation of NF-{kappa}B is induced by viral latent membrane protein 1 (LMP1).
Category
Cancer
BRITE hierarchy
Pathway
Transcriptional misregulation in cancers
Viral carcinogenesis
Gene
IKBA (mutation) [HSA:4792] [KO:K04734]
IKBE (mutation) [HSA:4794] [KO:K05872]
c-rel (amplification) [HSA:5966] [KO:K09254]
CD95 (mutation) [HSA:355] [KO:K04390]
Pathogen
EBV (Epstein-Barr Virus)
Marker
CD30 [HSA:943]
IL-10 [HSA:3586]
Drug
Doxorubicin (Adriamycin) [DG:DG00696]
Bleomycin [DG:DG00705]
Vinblastine [DG:DG00689]
Dacarbazine (ABVD) [DG:DG00680]
Comment
ICD-O: 9650/3, Tumor type: Classical Hodgkin lymphoma
ICD-O: 9663/3, Tumor type: Nodular sclerosis classical Hodgkin lymphoma
ICD-O: 9651/3, Tumor type: Lymphocyte-rich classical Hodgkin lymphoma
ICD-O: 9652/3, Tumor type: Mixed cellularity classical Hodgkin lymphoma
ICD-O: 9653/3, Tumor type: Lymphocyte-depleted classical Hodgkin lymphoma
Other DBs
ICD-10: 
MeSH: 
Reference
PMID:15618006 (tumor type)
  Authors
Kuppers R, Hansmann ML.
  Title
The Hodgkin and Reed/Sternberg cell.
  Journal
Int J Biochem Cell Biol 37:511-7 (2005)
Reference
PMID:15803153 (gene, carcinogen, tumor type)
  Authors
Kuppers R.
  Title
Mechanisms of B-cell lymphoma pathogenesis.
  Journal
Nat Rev Cancer 5:251-62 (2005)
Reference
PMID:16385563 (gene, carcinogen)
  Authors
Brauninger A, Schmitz R, Bechtel D, Renne C, Hansmann ML, Kuppers R.
  Title
Molecular biology of Hodgkin's and Reed/Sternberg cells in Hodgkin's lymphoma.
  Journal
Int J Cancer 118:1853-61 (2006)
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:12078906 (marker)
  Authors
Zander T, Wiedenmann S, Wolf J.
  Title
Prognostic factors in Hodgkin's lymphoma.
  Journal
Ann Oncol 13 Suppl 1:67-74 (2002)
Reference
PMID:11142480 (marker)
  Authors
Axdorph U, Sjoberg J, Grimfors G, Landgren O, Porwit-MacDonald A, Bjorkholm M.
  Title
Biological markers may add to prediction of outcome achieved by the International Prognostic Score in Hodgkin's disease.
  Journal
Ann Oncol 11:1405-11 (2000)
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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