KEGG   DISEASE: 慢性骨髄性白血病
エントリ  
H00004                                                             

名称    
慢性骨髄性白血病
概要    
Chronic myeloid leukemia (CML) is a clonal myeloproliferative disorder of a pluripotent stem cell. The natural history of CML has a triphasic clinical course comprising of an initial chronic phase (CP), which is characterized by expansion of functionally normal myeloid cells, followed by an accelerated phase (AP) and finally a more aggressive blast phase (BP), with loss of terminal differentiation capacity. On the cellular level, CML is associated with a specific chromosome abnormality, the t(9; 22) reciprocal translocation that forms the Philadelphia (Ph) chromosome. The Ph chromosome is the result of a molecular rearrangement between the c-ABL proto-oncogene on chromosome 9 and the BCR (breakpoint cluster region) gene on chromosome 22. The BCR/ABL fusion gene encodes p210 BCR/ABL, an oncoprotein, which, unlike the normal p145 c-Abl, has constitutive tyrosine kinase activity and is predominantly localized in the cytoplasm. While fusion of c-ABL and BCR is believed to be the primary cause of the chronic phase of CML, progression to blast crisis requires other molecular changes. Common secondary abnormalities include mutations in TP53, RB, and p16/INK4A, or overexpression of genes such as EVI1. Additional chromosome translocations are also observed,such as t(3;21)(q26;q22), which generates AML1-EVI1.
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
   H00004  慢性骨髄性白血病
ICD-11 による疾患分類 [BR:jp08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Myeloproliferative neoplasms
    2A20  Non mast cell myeloproliferative neoplasms
     H00004  慢性骨髄性白血病
パスウェイ 
hsa05220  慢性骨髄性白血病
関連パスウェイ
hsa05200  Pathways in cancer
ネットワーク
nt06276  Chronic myeloid leukemia
  エレメント
N00002  BCR-ABL fusion kinase to RAS-ERK signaling pathway
N00048  BCR-ABL fusion kinase to PI3K signaling pathway
N00055  BCR-ABL fusion kinase to Jak-STAT signaling pathway
N00067  Deleted p14(ARF) to p21-cell cycle G1/S
N00070  Mutation-inactivated p16(INK4a) to p16-cell cycle G1/S
N00074  Loss of RB1 to cell cycle G1/S
N00106  AML1-EVI1 fusion to TGF-beta signaling pathway
N00107  EVI-1 overexpression to TGF-beta signaling pathway
N00115  Mutation-inactivated TP53 to transcription
病因遺伝子 
BCR-ABL (translocation) [HSA:25] [KO:K06619]
EVI1 (overexpression) [HSA:2122] [KO:K04462]
AML1 (translocation) [HSA:861] [KO:K08367]
p16/INK4A (mutation) [HSA:1029] [KO:K06621]
p53 (mutation) [HSA:7157] [KO:K04451]
RB1 (mutation) [HSA:5925] [KO:K06618]
発癌物質  
1,3-Butadiene [CPD:C16450]
Rubber industry
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]
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酢酸エステル [DR:D00979]
メトトレキサート [DR:D00142]
シクロホスファミド水和物 [DR:D00287]
ブスルファン [DR:D00248]
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]
フルダラビンリン酸エステル [DR:D01907]
ヒドロキシカルバミド [DR:D00341]
マイトマイシンC [DR:D00208]
ダウノルビシン塩酸塩 [DR:D01264]
イダルビシン塩酸塩 [DR:D01747]
ビンデシン硫酸塩 [DR:D01769]
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]
ダサチニブ水和物 [DR:D06414]
ニロチニブ塩酸塩水和物 [DR:D06413]
ボスチニブ水和物 [DR:D09728]
ポナチニブ塩酸塩 [DR:D09951]
インターフェロンアルファ (NAMALWA) [DR:D03305]
コメント  
ICD-O: 9875/3, Tumor type: Chronic myelogenous leukaemia
リンク   
ICD-11: 2A20.0
ICD-10: C92.1
MeSH: D015464
文献    
PMID:15719031 (gene, tumor type)
  著者
Ren R.
  タイトル
Mechanisms of BCR-ABL in the pathogenesis of chronic myelogenous leukaemia.
  雑誌
Nat Rev Cancer 5:172-83 (2005)
DOI:10.1038/nrc1567
文献    
  著者
Faderl S, Talpaz M, Estrov Z, O'Brien S, Kurzrock R, Kantarjian HM.
  タイトル
The biology of chronic myeloid leukemia.
  雑誌
N Engl J Med 341:164-72 (1999)
DOI:10.1056/NEJM199907153410306
文献    
  著者
Calabretta B, Perrotti D.
  タイトル
The biology of CML blast crisis.
  雑誌
Blood 103:4010-22 (2004)
DOI:10.1182/blood-2003-12-4111
文献    
PMID:16155477 (carcinogen)
  著者
Graff JJ, Sathiakumar N, Macaluso M, Maldonado G, Matthews R, Delzell E.
  タイトル
Chemical exposures in the synthetic rubber industry and lymphohematopoietic cancer mortality.
  雑誌
J Occup Environ Med 47:916-32 (2005)
DOI:10.1097/01.jom.0000172866.16615.db
文献    
PMID:9498905 (carcinogen)
  著者
Blair A, Kazerouni N.
  タイトル
Reactive chemicals and cancer.
  雑誌
Cancer Causes Control 8:473-90 (1997)
DOI:10.1023/a:1018417623867
文献    
PMID:16873420 (carcinogen)
  著者
Alder N, Fenty J, Warren F, Sutton AJ, Rushton L, Jones DR, Abrams KR.
  タイトル
Meta-analysis of mortality and cancer incidence among workers in the synthetic rubber-producing industry.
  雑誌
Am J Epidemiol 164:405-20 (2006)
DOI:10.1093/aje/kwj252
文献    
PMID:16299089 (carcinogen)
  著者
Sathiakumar N, Graff J, Macaluso M, Maldonado G, Matthews R, Delzell E.
  タイトル
An updated study of mortality among North American synthetic rubber industry workers.
  雑誌
Occup Environ Med 62:822-9 (2005)
DOI:10.1136/oem.2004.018176
文献    
PMID:15265366 (marker)
  著者
Chen ZX, Kaeda J, Saunders S, Goldman JM.
  タイトル
Expression patterns of WT-1 and Bcr-Abl measured by TaqMan quantitative real-time RT-PCR during follow-up of leukemia patients with the Ph chromosome.
  雑誌
Chin Med J (Engl) 117:968-71 (2004)
文献    
PMID:15661271 (marker)
  著者
Na IK, Kreuzer KA, Lupberger J, Dorken B, le Coutre P.
  タイトル
Quantitative RT-PCR of Wilms tumor gene transcripts (WT1) for the molecular monitoring of patients with accelerated phase bcr/abl + CML.
  雑誌
Leuk Res 29:343-5 (2005)
DOI:10.1016/j.leukres.2004.08.003
文献    
PMID:11529849 (marker)
  著者
Kreuzer KA, Saborowski A, Lupberger J, Appelt C, Na IK, le Coutre P, Schmidt CA.
  タイトル
Fluorescent 5'-exonuclease assay for the absolute quantification of Wilms' tumour gene (WT1) mRNA: implications for monitoring human leukaemias.
  雑誌
Br J Haematol 114:313-8 (2001)
DOI:10.1046/j.1365-2141.2001.02912.x
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