KEGG   DISEASE: T細胞急性リンパ性白血病
エントリ  
H00002                                                             

名称    
T細胞急性リンパ性白血病;
T細胞リンパ芽球性白血病
  下位グループ
T細胞リンパ芽球性リンパ腫
概要    
Acute lymphocytic leukemia (ALL) is a clonal stem cell malignancy of excessive lymphoblast proliferation. It is now understood that ALL and lymphoblastic lymphoma are the same disease entities at the morphologic and immunophenotypic levels and classified as either B- and T-cell lymphoblastic leukemia/lymphoma (B-ALL and T-ALL). T-ALL comprises 15% of paediatric and 25% of adult ALL cases. T cell transformation is a multi-step process in which different genetic alterations cooperate to alter the normal mechanisms that control cell growth, proliferation, survival, and differentiation during thymocyte development. In this context, constitutive activation of NOTCH1 signaling is the most prominent oncogenic pathway in T cell transformation. In addition, T-ALLs characteristically show the translocation and aberrant expression of transcription factor oncogenes. These oncogenic transcription factors include T-cell leukaemia homeobox protein 1 (TLX1 also known as HOX11), TLX3 (HOX11L2), LYL1, TAL1 and MLL.
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
   H00002  T細胞急性リンパ性白血病
ICD-11 による疾患分類 [BR:jp08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Precursor lymphoid neoplasms
    2A71  Precursor T-lymphoblastic neoplasms
     H00002  T細胞急性リンパ性白血病
関連パスウェイ
hsa05202  Transcriptional misregulation in cancer
ネットワーク
  エレメント
N00120  MLL-ENL fusion to transcriptional activation
N00121  LMO2-rearrangement to transcriptional activation
N00122  LMO2-rearrangement to transcriptional repression
N00144  TLX1 rearrangement to transcriptional repression
病因遺伝子 
NOTCH1 (mutation) [HSA:4851] [KO:K02599]
TAL1 (overexpression) [HSA:6886] [KO:K09068]
LYL1 (expression) [HSA:4066] [KO:K15604]
MLL-ENL (translocation) [HSA:4297] [KO:K09186]
HOX11 (translocation) [HSA:3195] [KO:K09340]
MYC (translocation) [HSA:4609] [KO:K04377]
LMO2 (translocation) [HSA:4005] [KO:K15612]
HOX11L2 (translocation) [HSA:30012] [KO:K15607]
PICALM-MLLT10 (translocation) [HSA:8028] [KO:K23588]
発癌物質  
*Thorium-232 and its decay products, administered intravenously as a colloidal dispersion of thorium232 dioxide
*: Although this is a risk factor for ALL in general, the strength of association with different histological tumor types is not well understood.
治療薬   
ベタメタゾンリン酸エステルナトリウム [DR:D00972]
プレドニゾロン [DR:D00472]
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]
シクロホスファミド水和物 [DR:D00287]
イホスファミド [DR:D00343]
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]
メルカプトプリン水和物 [DR:D00161]
シタラビン [DR:D00168]
ゲムシタビン塩酸塩 [DR:D01155]
エノシタビン [DR:D01633]
クロファラビン [DR:D03546]
ネララビン [DR:D05134]
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]
ダウノルビシン塩酸塩 [DR:D01264]
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]
イノツズマブオゾガマイシン [DR:D08933] (CD22陽性)
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]
シスプラチン [DR:D00275]
カルボプラチン [DR:D01363]
L-アスパラギナーゼ [DR:D02997]
ソブゾキサン [DR:D01404]
コメント  
ICD-O-3.1: 9837/3 T lymphoblastic leukemia/lymphoma
リンク   
ICD-11: 2A71
ICD-10: C83.5 C91.0
MeSH: D054218
文献    
  著者
Roberts KG, Mullighan CG
  タイトル
Genomics in acute lymphoblastic leukaemia: insights and treatment implications.
  雑誌
Nat Rev Clin Oncol 12:344-57 (2015)
DOI:10.1038/nrclinonc.2015.38
文献    
  著者
Teitell MA, Pandolfi PP
  タイトル
Molecular genetics of acute lymphoblastic leukemia.
  雑誌
Annu Rev Pathol 4:175-98 (2009)
DOI:10.1146/annurev.pathol.4.110807.092227
文献    
  著者
Van Vlierberghe P, Ferrando A
  タイトル
The molecular basis of T cell acute lymphoblastic leukemia.
  雑誌
J Clin Invest 122:3398-406 (2012)
DOI:10.1172/JCI61269
文献    
  著者
Cornell RF, Palmer J
  タイトル
Adult acute leukemia.
  雑誌
Dis Mon 58:219-38 (2012)
DOI:10.1016/j.disamonth.2012.01.011
文献    
  著者
Kraszewska MD, Dawidowska M, Szczepanski T, Witt M
  タイトル
T-cell acute lymphoblastic leukaemia: recent molecular biology findings.
  雑誌
Br J Haematol 156:303-15 (2012)
DOI:10.1111/j.1365-2141.2011.08957.x
文献    
PMID:27069254 (tumor type)
  著者
Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, Bloomfield CD, Cazzola M, Vardiman JW
  タイトル
The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia.
  雑誌
Blood 127:2391-405 (2016)
DOI:10.1182/blood-2016-03-643544
文献    
PMID:12556326 (carcinogen)
  著者
Harrison JD, Muirhead CR.
  タイトル
Quantitative comparisons of cancer induction in humans by internally deposited radionuclides and external radiation.
  雑誌
Int J Radiat Biol 79:1-13 (2003)
DOI:10.1080/0955300021000038671

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