KEGG   DISEASE: 成人T細胞性白血病
エントリ  
H00009                                                             
名称    
成人T細胞性白血病
概要    
Adult T-cell leukemia (ATL) is one of the most aggressive hematologic malignancies and is caused by human T-cell leukemia virus type 1 (HTLV-1). The HTLV-1 Tax protein has been demonstrated to be the oncogenic protein of the virus. Tax may contribute to the process of carcinogenesis by a variety of mechanisms, including upregulating the expression of cellular genes involved in T cell growth and proliferation, including IL-2, IL-2R-alpha, and IL-15. However, ATL cells do not always need Tax expression in the later stage of leukemogenesis. Genetic and epigenetic changes should be implicated in such multistep leukemogenesis. Regarding genetic changes, mutation of p53, and deletion of p16 have been reported in ATL. Mutations of Fas gene are also reported in patients with ATL cells. However, such genetic changes were not frequently detected. In this regard, epigenetic change of p16/INK4A gene was more frequent in ATL cells, and accumulated according to the disease progression. This finding suggests that epigenetic change, including DNA methylation, plays an important role in the leukemogenesis of ATL.
カテゴリ  
がん; ウイルス感染症
階層分類  
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
   成熟T細胞またはNK細胞腫瘍
    2A90  成熟T細胞腫瘍, 特定型, 節性または全身性
     H00009  成人T細胞性白血病
パスウェイに基づく疾患分類 [BR:jp08402]
 シグナル伝達
  nt06507  TGFB シグナリング
   H00009  成人T細胞性白血病
  nt06518  JAK-STAT シグナリング
   H00009  成人T細胞性白血病
  nt06516  TNF シグナリング
   H00009  成人T細胞性白血病
 免疫系
  nt06537  TCR/BCR シグナリング
   H00009  成人T細胞性白血病
ゲノムに基づく感染症分類 [BR:jp08401]
 ウイルス感染症
  ssRNA-RTウイルスによる感染症
   H00009  成人T細胞性白血病
疾患パスウェイ
hsa05166  ヒトT細胞白血病ウイルス1感染
ネットワーク
nt06160 Human T-cell leukemia virus 1 (HTLV-1)
nt06507 TGFB signaling
nt06516 TNF signaling
nt06518 JAK-STAT signaling
nt06537 TCR/BCR signaling
病因遺伝子 
p53 (mutation) [HSA:7157] [KO:K04451]
p16/INK4A (deletion, promoter methylation) [HSA:1029] [KO:K06621]
Fas (mutation) [HSA:355] [KO:K04390]
病原体   
Human T-lymphotropic virus 1 [GN:T40003]
治療薬   
モガムリズマブ [DR:D09761] (CCR4陽性)
ベキサロテン [DR:D03106]
ツシジノスタット [DR:D10993]
レナリドミド水和物 [DR:D09813]
ソブゾキサン [DR:D01404]
バレメトスタットトシル酸塩 [DR:D11662]
リンク   
ICD-11: 2A90.5
ICD-10: C91.5
MeSH: D015459
文献    
  著者
Matsuoka M
  タイトル
Human T-cell leukemia virus type I and adult T-cell leukemia.
  雑誌
Oncogene 22:5131-40 (2003)
DOI:10.1038/sj.onc.1206551
文献    
  著者
Yasunaga J, Matsuoka M
  タイトル
Leukemogenesis of adult T-cell leukemia.
  雑誌
Int J Hematol 78:312-20 (2003)
DOI:10.1007/BF02983555
文献    
  著者
Maeda T, Yamada Y, Moriuchi R, Sugahara K, Tsuruda K, Joh T, Atogami S, Tsukasaki K, Tomonaga M, Kamihira S.
  タイトル
Fas gene mutation in the progression of adult T cell leukemia.
  雑誌
J Exp Med 189:1063-71 (1999)
DOI:10.1084/jem.189.7.1063
文献    
  著者
Yasunaga J, Matsuoka M.
  タイトル
Human T-cell leukemia virus type I induces adult T-cell leukemia: from clinical aspects to molecular mechanisms.
  雑誌
Cancer Control 14:133-40 (2007)
DOI:10.1177/107327480701400206
文献    
  著者
Jarrett RF.
  タイトル
Viruses and lymphoma/leukaemia.
  雑誌
J Pathol 208:176-86 (2006)
DOI:10.1002/path.1905
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