KEGG   DISEASE: B細胞急性リンパ性白血病
エントリ  
H00001                                                             

名称    
B細胞急性リンパ性白血病;
B細胞リンパ芽球性白血病
  下位グループ
フィラデルフィア染色体陽性急性リンパ性白血病
B細胞リンパ芽球性リンパ腫
  上位グループ
非ホジキンリンパ腫 [DS:H02418]
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). In the case of B-ALL, numerous reports have demonstrated that recurring genetic abnormalities are associated with sufficiently unique clinical, immunophenotypic, and/or prognostic features so that they can be considered as distinct entities. The most common rearrangements observed in B-ALL are the t(12;21) (p13;q22) rearrangement resulting in expression of the ETV6-RUNX1 fusion (TEL-AML1); the t(1;19) (q23;p13) translocation that results in expression of the TCF3 (E2A) fusion partner, (also known as TCF3) TFPT-PBX1 fusion (E2A-PBX); the t(9;22) (q34;q11.2) "Philadelphia" chromosome resulting in expression of the BCR-ABL1 fusion; and rearrangements of MLL (also known as KMT2A) at 11q23 to a diverse array of fusion partners. If none of these specific genetic abnormalities are found, the designation of "B lymphoblastic leukemia/lymphoma, not otherwise specified," is appropriate.
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
   H00001  B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
    2A70  Precursor B-lymphoblastic neoplasms
     H00001  B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
N00117  E2A-PBX1 fusion to transcriptional activation
N00118  TEL-AML1 fusion to transcriptional repression
N00119  MLL-AF4 fusion to transcriptional activation
病因遺伝子 
BCR-ABL (translocation) [HSA:25] [KO:K06619]
MLL-AF4 (translocation) [HSA:4297 4299] [KO:K09186 K15184]
E2A-PBX1 (translocation) [HSA:6929 5087] [KO:K09063 K09355]
TEL-AML1 (translocation) [HSA:861] [KO:K08367]
c-MYC (rearrangement) [HSA:4609] [KO:K04377]
CRLF2 (rearrangement) [HSA:64109] [KO:K05078]
PAX5 (rearrangement) [HSA:5079] [KO:K09383]
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]
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]
イマチニブメシル酸塩 [DR:D01441] (フィラデルフィア染色体陽性)
ダサチニブ水和物 [DR:D06414] (フィラデルフィア染色体陽性)
ポナチニブ塩酸塩 [DR:D09951] (フィラデルフィア染色体陽性)
シスプラチン [DR:D00275]
カルボプラチン [DR:D01363]
ブリナツモマブ [DR:D09325]
L-アスパラギナーゼ [DR:D02997]
ソブゾキサン [DR:D01404]
チサゲンレクルユーセル [DR:D11386] (CD19陽性)
コメント  
ICD-O-3.1: 9811/3 B lymphoblastic leukemia/lymphoma, NOS
ICD-O-3.1: 9812/3 B lymphoblastic leukemia/lymphoma with t(9;22)(q34;q11.2); BCR-ABL1
ICD-O-3.1: 9813/3 B lymphoblastic leukemia/lymphoma with t(v;11q23); MLL rearranged
ICD-O-3.1: 9814/3 B lymphoblastic leukemia/lymphoma with t(12;21)(p13;q22); TEL-AML1 (ETV6-RUNX1)
ICD-O-3.1: 9815/3 B lymphoblastic leukemia/lymphoma with hyperdiploidy
ICD-O-3.1: 9816/3 B lymphoblastic leukemia/lymphoma with hypodiploidy (Hypodiploid ALL)
ICD-O-3.1: 9817/3 B lymphoblastic leukemia/lymphoma with t(5;14)(q31;q32); IL3-IGH
ICD-O-3.1: 9818/3 B lymphoblastic leukemia/lymphoma with t(1;19)(q23;p13.3); E2A-PBX1 (TCF3-PBX1)
リンク   
ICD-11: 2A70
ICD-10: C83.5 C91.0
MeSH: D054198
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
Mullighan CG
  タイトル
Molecular genetics of B-precursor acute lymphoblastic leukemia.
  雑誌
J Clin Invest 122:3407-15 (2012)
DOI:10.1172/JCI61203
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
Vardiman JW, Thiele J, Arber DA, Brunning RD, Borowitz MJ, Porwit A, Harris NL, Le Beau MM, Hellstrom-Lindberg E, Tefferi A, Bloomfield CD
  タイトル
The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes.
  雑誌
Blood 114:937-51 (2009)
DOI:10.1182/blood-2009-03-209262
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    
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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