KEGG   DISEASE: 末梢性T細胞リンパ腫
エントリ  
H01892                                                             

名称    
末梢性T細胞リンパ腫
  下位グループ
未分化大細胞リンパ腫 [DS:H01601]
  上位グループ
非ホジキンリンパ腫 [DS:H02418]
概要    
Peripheral T cell lymphomas (PTCLs) are a group of rare lymphomas originating from mature (i.e., post-thymic or "peripheral") T lymphocytes and NK cells. With regard to the current WHO classification, leukemic, cutaneous, nodal, and extranodal PTCL are distinguished. The most common subtypes worldwide are the nodal T cell lymphomas. In the nodal T cell lymphomas, the major subtypes are PTCL, not otherwise specified (PTCL-NOS), angioimmunoblastic T cell lymphoma (AITL), anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL) and ALK-negative ALCL. Novel approaches are gradually clarifying the molecular pathogenesis of PTCLs. ALK gene translocation and dual specificity phosphatase 22 (DUSP22) gene translocation are seen in ALK-positive ALCL and ALK-negative ALCL, respectively. Recently, gene mutations in epigenetic regulators, including Ten-Eleven Translocation 2 (TET2), DNA methyltransferase 3A (DNMT3A), and isocitrate dehydrogenase 1 /2 (IDH1/2), are discovered in hematologic malignancies. The mutation frequencies were especially high in AITL and PTCL-NOS.
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
   H01892  末梢性T細胞リンパ腫
ICD-11 による疾患分類 [BR:jp08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Mature T-cell or NK-cell neoplasms
    2A90  Mature T-cell lymphoma, specified types, nodal or systemic
     H01892  末梢性T細胞リンパ腫
関連パスウェイ
hsa05200  Pathways in cancer
hsa05230  Central carbon metabolism in cancer
病因遺伝子 
NPM-ALK (translocation) [HSA:238] [KO:K05119]
DUSP22-IRF4 (translocation) [HSA:56940] [KO:K14165]
TET2 (mutation) [HSA:54790] [KO:K24309]
DNMT3A (mutation) [HSA:1788] [KO:K17398]
IDH1 (mutation) [HSA:3417] [KO:K00031]
IDH2 (mutation) [HSA:3418] [KO:K00031]
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]
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]
シタラビン [DR:D00168]
ゲムシタビン塩酸塩 [DR:D01155]
プララトレキサート [DR:D05589]
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]
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]
ビンブラスチン硫酸塩 [DR:D01068]
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]
ブレンツキシマブベドチン [DR:D09587] (CD30陽性)
モガムリズマブ [DR:D09761] (CCR4陽性)
ロミデプシン [DR:D06637]
L-アスパラギナーゼ [DR:D02997]
デニロイキンジフチトクス [DR:D03682]
ソブゾキサン [DR:D01404]
フォロデシン塩酸塩 [DR:D04245]
コメント  
9702/3 Mature T-cell lymphoma, NOS
9705/3 Angioimmunoblastic T-cell lymphoma
9714/3 Anaplastic large cell lymphoma, T cell and Null cell type
リンク   
ICD-11: 2A90
ICD-10: C84.4 C84.6 C84.7 C86.5
MeSH: D016411
文献    
  著者
Sandell RF, Boddicker RL, Feldman AL
  タイトル
Genetic Landscape and Classification of Peripheral T Cell Lymphomas.
  雑誌
Curr Oncol Rep 19:28 (2017)
DOI:10.1007/s11912-017-0582-9
文献    
  著者
Sakata-Yanagimoto M, Chiba S
  タイトル
Molecular Pathogenesis of Peripheral T Cell Lymphoma.
  雑誌
Curr Hematol Malig Rep 10:429-37 (2015)
DOI:10.1007/s11899-015-0289-7
文献    
  著者
Reimer P
  タイトル
New developments in the treatment of peripheral T-cell lymphoma - role of Belinostat.
  雑誌
Cancer Manag Res 7:145-51 (2015)
DOI:10.2147/CMAR.S85351
文献    
  著者
Sakata-Yanagimoto M
  タイトル
Multistep tumorigenesis in peripheral T cell lymphoma.
  雑誌
Int J Hematol 102:523-7 (2015)
DOI:10.1007/s12185-015-1738-8
文献    
PMID:28622960 (drug)
  著者
Feldman T, Farber CM, Choi K, Faria C, Goy A, Connors J, Paramanathan D, Kaur S, Schultz E, McGuire M, Goldberg SL
  タイトル
Treatment of Peripheral T-Cell Lymphoma in Community Settings.
  雑誌
Clin Lymphoma Myeloma Leuk 17:354-361 (2017)
DOI:10.1016/j.clml.2017.05.001
文献    
PMID:27071634 (drug)
  著者
Zhang Y, Xu W, Liu H, Li J
  タイトル
Therapeutic options in peripheral T cell lymphoma.
  雑誌
J Hematol Oncol 9:37 (2016)
DOI:10.1186/s13045-016-0267-0
文献    
PMID:28115372 (drug)
  著者
Broccoli A, Zinzani PL
  タイトル
Peripheral T-cell lymphoma, not otherwise specified.
  雑誌
Blood 129:1103-1112 (2017)
DOI:10.1182/blood-2016-08-692566

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