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
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
   H00009  成人T細胞性白血病
感染症疾患 [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
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 
hsa05166  ヒトT細胞白血病ウイルス1感染
関連パスウェイ
hsa05203  Viral carcinogenesis
hsa05200  Pathways in cancer
ネットワーク
nt06114  PI3K signaling (viruses)
nt06118  TGFB signaling (viruses)
nt06119  JAK-STAT signaling (viruses)
nt06120  Calcium signaling (viruses and bacteria)
nt06123  TNF signaling (viruses and bacteria)
nt06129  MHC presentation (viruses)
nt06130  Cell cycle (viruses)
nt06140  Transcription (viruses)
nt06150  Cytokine-cytokine receptor interaction (viruses)
nt06160  Human T-cell leukemia virus 1 (HTLV-1)
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)
ペントスタチン [DR:D00155]
レナリドミド水和物 [DR:D09813]
ソブゾキサン [DR:D01404]
ツシジノスタット [DR:D10993]
コメント  
ICD-O: 9827/3, Tumor type: Adult T-cell leukaemia
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    
PMID:10190897 (gene, tumor type)
  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    
PMID:17387298 (carcinogen)
  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    
PMID:16362996 (carcinogen)
  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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