KEGG   DISEASE: ホジキンリンパ腫
エントリ  
H00007                                                             
名称    
ホジキンリンパ腫
概要    
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).
カテゴリ  
がん
階層分類  
ヒト疾患 [BR:jp08402]
 がん
  造血組織およびリンパ系組織のがん
   H00007  ホジキンリンパ腫
ICD-11 による疾患分類 [BR:jp08403]
 02 腫瘍
  造血またはリンパ組織の腫瘍
   2B30  ホジキンリンパ腫
    H00007  ホジキンリンパ腫
関連パスウェイ
hsa05200  Pathways in cancer
hsa05202  Transcriptional misregulation in cancer
hsa05169  Epstein-Barr virus infection
hsa05203  Viral carcinogenesis
病因遺伝子 
NFKBIA [HSA:4792] [KO:K04734]
NFKBIE [HSA:4794] [KO:K05872]
REL (amplification) [HSA:5966] [KO:K09254]
FAS [HSA:355] [KO:K04390]
KLHDC8B [HSA:200942]
病原体   
Human herpesvirus 4 (Epstein-Barr virus) [GN:T40079]
治療薬   
デキサメタゾン [DR:D00292]
デキサメタゾンリン酸エステルナトリウム [DR:D00975]
ベタメタゾンリン酸エステルナトリウム [DR:D00972]
トリアムシノロンアセトニド [DR:D00983]
ヒドロコルチゾン [DR:D00088]
ヒドロコルチゾンコハク酸エステルナトリウム [DR:D00978]
コルチゾン酢酸エステル [DR:D00973]
トリアムシノロン [DR:D00385]
ベタメタゾン [DR:D00244]
プレドニゾロン [DR:D00472]
プレドニゾロンコハク酸エステルナトリウム [DR:D01239]
メチルプレドニゾロン [DR:D00407]
メチルプレドニゾロンコハク酸エステルナトリウム [DR:D00751]
メチルプレドニゾロン酢酸エステル [DR:D00979]
イホスファミド [DR:D00343]
ブスルファン [DR:D00248]
メルファラン [DR:D00369]
ニムスチン塩酸塩 [DR:D01059]
ラニムスチン [DR:D01760]
ダカルバジン [DR:D00288]
シタラビン [DR:D00168]
ゲムシタビン塩酸塩 [DR:D01155]
ブレオマイシン塩酸塩 [DR:D03229]
ペプロマイシン硫酸塩 [DR:D02063]
ドキソルビシン塩酸塩 [DR:D01275]
エピルビシン塩酸塩 [DR:D02214]
アクラルビシン塩酸塩 [DR:D01911]
ピラルビシン塩酸塩 [DR:D08386]
ビンブラスチン硫酸塩 [DR:D01068]
ビンクリスチン硫酸塩 [DR:D02197]
ビンデシン硫酸塩 [DR:D01769]
エトポシド [DR:D00125]
ミトキサントロン塩酸塩 [DR:D02166]
ニボルマブ [DR:D10316]
ペムブロリズマブ [DR:D10574]
ブレンツキシマブベドチン [DR:D09587] (CD30陽性)
シスプラチン [DR:D00275]
カルボプラチン [DR:D01363]
プロカルバジン塩酸塩 [DR:D00478]
L-アスパラギナーゼ [DR:D02997]
ソブゾキサン [DR:D01404]
リンク   
ICD-11: 2B30
ICD-10: C81
MeSH: D006689
OMIM: 236000
文献    
PMID:15618006 (NFKBIA, NFKBIE, REL, FAS)
  著者
Kuppers R, Hansmann ML.
  タイトル
The Hodgkin and Reed/Sternberg cell.
  雑誌
Int J Biochem Cell Biol 37:511-7 (2005)
DOI:10.1016/j.biocel.2003.10.025
文献    
PMID:15803153 (NFKBIA, NFKBIE, REL, FAS)
  著者
Kuppers R.
  タイトル
Mechanisms of B-cell lymphoma pathogenesis.
  雑誌
Nat Rev Cancer 5:251-62 (2005)
DOI:10.1038/nrc1589
文献    
PMID:19706467 (KLHDC8B)
  著者
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
  タイトル
Mutations in a gene encoding a midbody kelch protein in familial and sporadic classical Hodgkin lymphoma lead to binucleated cells.
  雑誌
Proc Natl Acad Sci U S A 106:14920-5 (2009)
DOI:10.1073/pnas.0904231106
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