KEGG   DISEASE: 有毛細胞白血病
エントリ  
H00006                                                             

名称    
有毛細胞白血病;
ヘアリー細胞白血病;
ヘアリーセル白血病
  上位グループ
非ホジキンリンパ腫 [DS:H02418]
概要    
Hairy cell Leukemia (HCL) is a chronic lymphoproliferative disorder that is defined, according to the WHO classification, as a mature (peripheral) B-cell neoplasm. HCL accounts for between 2-3% of all leukemia cases, with about 600 new cases diagnosed in the U.S. each year. With regard to genetic abnormalities of potential pathogenetic importance in HCL, only a few have been described. Mutations of p53 and Bcl-6 have been reported in about one-third of cases, but their functional significance is unclear. Over-expression of cyclin D1, an important cell cycle regulator, has been reported in cases of HCL as well as mantle cell lymphoma (MCL) and in a few cases of chronic lymphocytic leukemia among lymphoid malignancies.
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
   H00006  有毛細胞白血病
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B細胞腫瘍
    2A82  白血病的振る舞いを伴う成熟B細胞腫瘍
     H00006  有毛細胞白血病
関連パスウェイ
hsa05200  Pathways in cancer
hsa05202  Transcriptional misregulation in cancer
病因遺伝子 
p53 (mutation) [HSA:7157] [KO:K04451]
Bcl-6 (mutation) [HSA:604] [KO:K15618]
Cyclin D1 (overexpression) [HSA:595] [KO:K04503]
治療薬   
クラドリビン [DR:D01370]
リツキシマブ [DR:D02994] (CD20陽性)
ペントスタチン [DR:D00155]
インターフェロンアルファ (NAMALWA) [DR:D03305]
コメント  
ICD-O: 9940/3, Tumor type: Hairy cell leukemia
リンク   
ICD-11: 2A82.2
ICD-10: C91.4
MeSH: D007943
文献    
PMID:12670461 (gene, tumor type)
  著者
Zuzel M, Cawley JC.
  タイトル
The biology of hairy cells.
  雑誌
Best Pract Res Clin Haematol 16:1-13 (2003)
DOI:10.1016/S1521-6926(02)00082-8
文献    
(drug)
  著者
Papadakis MA and McPhee SJ |(ed)
  タイトル
Current Medical Diagnosis & Treatment 2015, Fifty-Fourth Edition
  雑誌
McGraw Hill Education
LinkDB    

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