KEGG   DISEASE: 多発性骨髄腫
エントリ  
H00010                                                             

名称    
多発性骨髄腫
概要    
Multiple myeloma is a disorder in which malignant plasma cells accumulate, generally derived from one clone in the bone marrow. Intricate interactions occur between the bone-marrow microenvironment and the myeloma cells, frequently causing bone destruction, which in turn stimulates tumor growth. Often it is preceded by a premalignant tumor called monoclonal gammopathy of undetermined significance (MGUS). Multiple oncogenic events have been identified that have contributed to the pathogenesis of myeloma. Among the earliest genetic events are translocations of the immunoglobulin heavy-chain gene locus, which leads to dysregulation of oncogenes at translocation partner regions (cyclin D1 at 11q13, FGFR3/MMSET at 4p16.3, c-MAF at 16q23, and cyclin D3 at 6p21), and deletions of 13q14, the site of a putative tumor suppressor gene. Additional molecular events include epigenetic changes and activation of oncogenes (mutations of N-RAS and K-RAS, and changes in c-MYC), which are usually associated with disease progression.
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
   H00010  多発性骨髄腫
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
    2A83  形質細胞腫瘍
     H00010  多発性骨髄腫
関連パスウェイ
hsa05200  Pathways in cancer
hsa05202  Transcriptional misregulation in cancer
ネットワーク
  エレメント
N00124  IGH-MAF fusion to transcriptional activation
N00125  IGH-MMSET fusion to transcriptional activation
N01398  Pentachlorophenol to CYP-mediated metabolism
病因遺伝子 
CCND1-IgH (translocation) [HSA:595] [KO:K04503]
CCND3-IgH (translocation) [HSA:896] [KO:K10152]
IgH-FGFR3 (translocation) [HSA:2261] [KO:K05094]
IgH-MMSET (translocation) [HSA:7468] [KO:K11424]
IgH-MAF (translocation) [HSA:4094] [KO:K09035]
N-ras (activating mutation) [HSA:4893] [KO:K07828]
K-ras (activating mutation) [HSA:3845] [KO:K07827]
c-MYC (dysregulation) [HSA:4609] [KO:K04377]
p53 (mutation) [HSA:7157] [KO:K04451]
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]
プレドニゾロン [DR:D00472]
ゾレドロン酸水和物 [DR:D01968]
デノスマブ [DR:D03684]
シクロホスファミド水和物 [DR:D00287]
メルファラン [DR:D00369]
ラニムスチン [DR:D01760]
フルダラビンリン酸エステル [DR:D01907]
ドキソルビシン塩酸塩 [DR:D01275]
ビンクリスチン硫酸塩 [DR:D02197]
エロツズマブ [DR:D09337]
ダラツムマブ [DR:D10777]
イサツキシマブ [DR:D11050]
ボルテゾミブ [DR:D03150]
カルフィルゾミブ [DR:D08880]
イキサゾミブクエン酸エステル [DR:D10131]
パノビノスタット乳酸塩 [DR:D10019]
サリドマイド [DR:D00754]
レナリドミド水和物 [DR:D09813]
ポマリドミド [DR:D08976]
ダラツムマブ・ボルヒアルロニダーゼアルファ [DR:D11967]
インターフェロンアルファ (NAMALWA) [DR:D03305]
コメント  
ICD-O: 9732/3, Tumor type: Plasma cell myeloma
リンク   
ICD-11: 2A83.1
ICD-10: C90.0
MeSH: D009101
文献    
  著者
Sirohi B, Powles R
  タイトル
Multiple myeloma.
  雑誌
Lancet 363:875-87 (2004)
DOI:10.1016/S0140-6736(04)15736-X
文献    
  著者
Seidl S, Kaufmann H, Drach J
  タイトル
New insights into the pathophysiology of multiple myeloma.
  雑誌
Lancet Oncol 4:557-64 (2003)
DOI:10.1016/S1470-2045(03)01195-1
文献    
  著者
Joy Ho P
  タイトル
Chromosomal and genetic abnormalities in myeloma.
  雑誌
Clin Lab Haematol 24:259-69 (2002)
DOI:10.1046/j.1365-2257.2002.00456.x
文献    
PMID:16155016 (gene, tumor type)
  著者
Bergsagel PL, Kuehl WM.
  タイトル
Molecular pathogenesis and a consequent classification of multiple myeloma.
  雑誌
J Clin Oncol 23:6333-8 (2005)
DOI:10.1200/JCO.2005.05.021
文献    
  著者
Podczaski E, Cain J.
  タイトル
Multiple myeloma.
  雑誌
Clin Obstet Gynecol 45:928-38 (2002)
DOI:10.1097/00003081-200209000-00038
文献    
PMID:9414264
  著者
Hallek M, Bergsagel PL, Anderson KC.
  タイトル
Multiple myeloma: increasing evidence for a multistep transformation process.
  雑誌
Blood 91:3-21 (1998)
文献    
  著者
Bergsagel PL, Kuehl WM
  タイトル
Chromosome translocations in multiple myeloma.
  雑誌
Oncogene 20:5611-22 (2001)
DOI:10.1038/sj.onc.1204641
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