KEGG   DISEASE: Hodgkin lymphomaHelp
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
Human diseases [BR:br08402]
 Cancers
  Cancers of haematopoietic and lymphoid tissues
   H00007  Hodgkin lymphoma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Mature T-cell or NK-cell neoplasms
    Mature T-cell or NK-cell lymphoma, primary cutaneous specified types
     2B30  Hodgkin lymphoma
      H00007  Hodgkin lymphoma
BRITE hierarchy
Pathway
hsa05202  Transcriptional misregulation in cancer
hsa05203  Viral carcinogenesis
hsa05169  Epstein-Barr virus infection
Network
  Element
N00123  Amplified REL to transcription
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
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
Human herpesvirus 4 (Epstein-Barr virus) [GN:T40079]
Drug
Cyclophosphamide [DR:D00287]
Chlorambucil [DR:D00266]
Mechlorethamine hydrochloride [DR:D04872]
Carmustine [DR:D00254]
Lomustine [DR:D00363]
Dacarbazine [DR:D00288]
Vinblastine sulfate [DR:D01068]
Doxorubicin hydrochloride [DR:D01275]
Bleomycin sulfate [DR:D02177]
Procarbazine hydrochloride [DR:D00478]
Brentuximab vedotin [DR:D09587]
Nivolumab [DR:D10316]
Pembrolizumab [DR:D10574]
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-11: 2B30
ICD-10: C81
MeSH: D006689
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)
DOI:10.1016/j.biocel.2003.10.025
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)
DOI:10.1038/nrc1589
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)
DOI:10.1002/ijc.21716
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: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)
DOI:10.1093/annonc/mdf613
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)
DOI:10.1023/A:1026551727795
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