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H00007                      Disease                                

Hodgkin lymphoma
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).
Human diseases [BR:br08402]
  Cancers of haematopoietic and lymphoid tissues
   H00007  Hodgkin 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
   C81  Hodgkin's disease
    H00007  Hodgkin lymphoma
BRITE hierarchy
Transcriptional misregulation in cancers
Viral carcinogenesis
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]
EBV (Epstein-Barr Virus)
CD30 [HSA:943]
IL-10 [HSA:3586]
Doxorubicin (Adriamycin) [DG:DG00696]
Bleomycin [DG:DG00705]
Vinblastine [DG:DG00689]
Dacarbazine (ABVD) [DG:DG00680]
Mechlorethamine [DR:D07671]
Etoposide [DG:DG00693]
Vincristine [DG:DG00690]
Prednisone [DG:DG00094]
Cyclophosphamide [DG:DG00675]
Procarbazine [DG:DG00706]
Prednisone [DG:DG00094]
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
PMID:15618006 (tumor type)
Kuppers R, Hansmann ML.
The Hodgkin and Reed/Sternberg cell.
Int J Biochem Cell Biol 37:511-7 (2005)
PMID:15803153 (gene, carcinogen, tumor type)
Kuppers R.
Mechanisms of B-cell lymphoma pathogenesis.
Nat Rev Cancer 5:251-62 (2005)
PMID:16385563 (gene, carcinogen)
Brauninger A, Schmitz R, Bechtel D, Renne C, Hansmann ML, Kuppers R.
Molecular biology of Hodgkin's and Reed/Sternberg cells in Hodgkin's lymphoma.
Int J Cancer 118:1853-61 (2006)
PMID:15760280 (carcinogen)
Schottenfeld D, Beebe-Dimmer JL.
Advances in cancer epidemiology: understanding causal mechanisms and the evidence for implementing interventions.
Annu Rev Public Health 26:37-60 (2005)
PMID:12078906 (marker)
Zander T, Wiedenmann S, Wolf J.
Prognostic factors in Hodgkin's lymphoma.
Ann Oncol 13 Suppl 1:67-74 (2002)
PMID:11142480 (marker)
Axdorph U, Sjoberg J, Grimfors G, Landgren O, Porwit-MacDonald A, Bjorkholm M.
Biological markers may add to prediction of outcome achieved by the International Prognostic Score in Hodgkin's disease.
Ann Oncol 11:1405-11 (2000)
Papadakis MA, McPhee SJ (ed).
Current Medical Diagnosis & Treatment 2015, Fifty-Fourth Edition
The McGraw-Hill Companies, Inc. (2014)

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