KEGG   DISEASE: 急性前骨髄球性白血病
エントリ  
H02542                                                             
名称    
急性前骨髄球性白血病
  上位グループ
急性骨髄性白血病 [DS:H00003]
概要    
Acute promyelocytic leukemia (APL) is the most malignant form of acute myeloid leukemia (AML) with a severe bleeding tendency and a fatal course of only weeks. Morphologically, it is identified as AML-M3 by the FAB classification. APL is characterized by a balanced reciprocal translocation between chromosomes 15 and 17, which results in the fusion between the promyelocytic leukemia (PML) gene and retinoic acid receptor alpha (RARA). The majority of APL patients present with the classic PML-RARA translocation. However, approximately 5% of patients with APL have a non- characteristic translocation. Other chimeric proteins have been observed including PLZF-RARA, NUMA1-RARA, and so on.
カテゴリ  
がん
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
   2A60  急性骨髄性白血病及び関連した前駆体の腫瘍
    H02542  急性前骨髄球性白血病
病因遺伝子 
PML-RARA (translocation) [HSA:5371 5914] [KO:K10054 K08527]
PLZF-RARA (translocation) [HSA:7704] [KO:K10055]
NUMA1-RARA (translocation) [HSA:4926] [KO:K16808]
治療薬   
三酸化二ヒ素 [DR:D02106]
リンク   
ICD-11: 2A60.0
MeSH: D015473
OMIM: 612376
文献    
  著者
Jimenez JJ, Chale RS, Abad AC, Schally AV
  タイトル
Acute promyelocytic leukemia (APL): a review of the literature.
  雑誌
Oncotarget 11:992-1003 (2020)
DOI:10.18632/oncotarget.27513
文献    
  著者
Wang ZY, Chen Z
  タイトル
Acute promyelocytic leukemia: from highly fatal to highly curable.
  雑誌
Blood 111:2505-15 (2008)
DOI:10.1182/blood-2007-07-102798
文献    
PMID:8387545
  著者
Chen SJ, Zelent A, Tong JH, Yu HQ, Wang ZY, Derre J, Berger R, Waxman S, Chen Z
  タイトル
Rearrangements of the retinoic acid receptor alpha and promyelocytic leukemia zinc finger genes resulting from t(11;17)(q23;q21) in a patient with acute promyelocytic leukemia.
  雑誌
J Clin Invest 91:2260-7 (1993)
DOI:10.1172/JCI116453
文献    
PMID:9288109
  著者
Wells RA, Catzavelos C, Kamel-Reid S
  タイトル
Fusion of retinoic acid receptor alpha to NuMA, the nuclear mitotic apparatus protein, by a variant translocation in acute promyelocytic leukaemia.
  雑誌
Nat Genet 17:109-13 (1997)
DOI:10.1038/ng0997-109
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