KEGG   DISEASE: Hodgkin lymphoma
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
   2B30  Hodgkin lymphoma
    H00007  Hodgkin lymphoma
Related
pathway
hsa05200  Pathways in cancer
hsa05202  Transcriptional misregulation in cancer
hsa05169  Epstein-Barr virus infection
hsa05203  Viral carcinogenesis
Gene
NFKBIA [HSA:4792] [KO:K04734]
NFKBIE [HSA:4794] [KO:K05872]
REL (amplification) [HSA:5966] [KO:K09254]
FAS [HSA:355] [KO:K04390]
KLHDC8B [HSA:200942]
Pathogen
Human herpesvirus 4 (Epstein-Barr virus) [GN:T40079]
Drug
Cyclophosphamide [DR:D00287]
Chlorambucil [DR:D00266]
Carmustine [DR:D00254]
Lomustine [DR:D00363]
Dacarbazine [DR:D00288]
Vinblastine sulfate [DR:D01068]
Vincristine sulfate [DR:D02197]
Doxorubicin hydrochloride [DR:D01275]
Bleomycin sulfate [DR:D02177]
Nivolumab [DR:D10316]
Pembrolizumab [DR:D10574]
Brentuximab vedotin [DR:D09587]
Procarbazine hydrochloride [DR:D00478]
Other DBs
ICD-11: 2B30
ICD-10: C81
MeSH: D006689
OMIM: 236000
Reference
PMID:15618006 (NFKBIA, NFKBIE, REL, FAS)
  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 (NFKBIA, NFKBIE, REL, FAS)
  Authors
Kuppers R.
  Title
Mechanisms of B-cell lymphoma pathogenesis.
  Journal
Nat Rev Cancer 5:251-62 (2005)
DOI:10.1038/nrc1589
Reference
PMID:19706467 (KLHDC8B)
  Authors
Salipante SJ, Mealiffe ME, Wechsler J, Krem MM, Liu Y, Namkoong S, Bhagat G, Kirchhoff T, Offit K, Lynch H, Wiernik PH, Roshal M, McMaster ML, Tucker M, Fromm JR, Goldin LR, Horwitz MS
  Title
Mutations in a gene encoding a midbody kelch protein in familial and sporadic classical Hodgkin lymphoma lead to binucleated cells.
  Journal
Proc Natl Acad Sci U S A 106:14920-5 (2009)
DOI:10.1073/pnas.0904231106
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