KEGG   DISEASE: びまん性大細胞型B細胞リンパ腫、非特定
エントリ  
H02434                                                             
名称    
びまん性大細胞型B細胞リンパ腫、非特定
  上位グループ
非ホジキンリンパ腫 [DS:H02418]
概要    
Diffuse large B-cell lymphoma, not otherwise specified (DLBCL, NOS) is the most common type of DLBCL. DLBCL accounts for 30-40% of all non-Hodgkin lymphomas (NHL), making it the most prevalent form of NHL. DLBCL can occur as primary disease or through histologic transformation from other low-grade B-cell lymphoma, and is a clinically and genetically heterogeneous disease. The cell-of-origin (COO) determination based on gene expression profiling (GEP) subdivides most DLBCL, NOS patients into two main categories, namely germinal center B-cell-like (GCB) and activated B-cell-like (ABC) DLBCL. GCB subtype is characterized by frequent mutations in involved histone methylation or acetylation (EZH2, EP300), B-cell homing (GNA13, GNAI2), PI3K pathway signalling (PTEN), and apoptotic pathway (BCL2). In contrast, ABC subtype is driven by frequent mutations in the B cell receptor and NFKB pathways (CARD11, CD79a/CD79b, TNFAIP3 and MYD88).
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
   成熟B細胞腫瘍
    2A81  びまん性大細胞型B細胞リンパ腫
     H02434  びまん性大細胞型B細胞リンパ腫、非特定
病因遺伝子 
EZH2 (mutation) [HSA:2146] [KO:K11430]
CREBBP (mutation/deletion) [HSA:1387] [KO:K04498]
EP300 (mutation/deletion) [HSA:2033] [KO:K04498]
GNA13 (mutation) [HSA:10672] [KO:K04639]
GNAI2 (mutation) [HSA:2768] [KO:K04346]
TNFRSF14 (mutation) [HSA:8764] [KO:K05152]
BCL6 (mutation) [HSA:604] [KO:K15618]
MYC (chromosomal translocation) [HSA:4609] [KO:K04377]
PTEN (deletion) [HSA:5728] [KO:K01110]
BCL2 (chromosomal translocation / mutation) [HSA:596] [KO:K02161]
CARD11 (mutation) [HSA:84433] [KO:K07367]
CD79A (mutation) [HSA:973] [KO:K06506]
CD79B (mutation) [HSA:974] [KO:K06507]
TNFAIP3 (mutation/deletion) [HSA:7128] [KO:K11859]
MYD88 (mutation) [HSA:4615] [KO:K04729]
CDKN2A (deletion) [HSA:1029] [KO:K06621]
CDKN2B (deletion) [HSA:1030] [KO:K04685]
PRDM1 (mutation/deletion) [HSA:639] [KO:K24501]
MLL2 (mutation) [HSA:8085] [KO:K09187]
MLL3 (mutation) [HSA:58508] [KO:K09188]
B2M (mutation/deletion) [HSA:567] [KO:K08055]
CD58 (mutation/deletion) [HSA:965] [KO:K06492]
TP53 (mutation) [HSA:7157] [KO:K04451]
MEF2B (mutation) [HSA:100271849] [KO:K09261]
FOXO1 (mutation) [HSA:2308] [KO:K07201]
治療薬   
ベンダムスチン塩酸塩 [DR:D07085]
ベンダムスチン塩酸塩水和物 [DR:D11661]
ポラツズマブベドチン [DR:D10761]
エプコリタマブ [DR:D12368]
リソカブタゲンマラルユーセル [DR:D11990]
リンク   
ICD-11: 2A81.Z
ICD-10: C83.3
MeSH: D016403
文献    
  著者
Li S, Young KH, Medeiros LJ
  タイトル
Diffuse large B-cell lymphoma.
  雑誌
Pathology 50:74-87 (2018)
DOI:10.1016/j.pathol.2017.09.006
文献    
  著者
Chiappella A, Crombie J, Guidetti A, Vitolo U, Armand P, Corradini P
  タイトル
Are We Ready to Treat Diffuse Large B-cell and High-Grade Lymphoma According to Major Genetic Subtypes?
  雑誌
Hemasphere 3:e284 (2019)
DOI:10.1097/HS9.0000000000000284
文献    
  著者
Ennishi D, Hsi ED, Steidl C, Scott DW
  タイトル
Toward a New Molecular Taxonomy of Diffuse Large B-cell Lymphoma.
  雑誌
Cancer Discov 2159-8290.CD-20-0174 (2020)
DOI:10.1158/2159-8290.CD-20-0174
文献    
  著者
Ohmachi K
  タイトル
JSH practical guidelines for hematological malignancies, 2018: II. Lymphoma-5-diffuse large B-cell lymphoma, not otherwise specified (DLBCL, NOS).
  雑誌
Int J Hematol 110:131-146 (2019)
DOI:10.1007/s12185-019-02681-3
文献    
  著者
Chapuy B, Stewart C, Dunford AJ, Kim J, Kamburov A, Redd RA, Lawrence MS, Roemer MGM, Li AJ, Ziepert M, Staiger AM, Wala JA, Ducar MD, Leshchiner I, Rheinbay E, Taylor-Weiner A, Coughlin CA, Hess JM, Pedamallu CS, Livitz D, Rosebrock D, Rosenberg M, Tracy AA, Horn H, van Hummelen P, Feldman AL, Link BK, Novak AJ, Cerhan JR, Habermann TM, Siebert R, Rosenwald A, Thorner AR, Meyerson ML, Golub TR, Beroukhim R, Wulf GG, Ott G, Rodig SJ, Monti S, Neuberg DS, Loeffler M, Pfreundschuh M, Trumper L, Getz G, Shipp MA
  タイトル
Molecular subtypes of diffuse large B cell lymphoma are associated with distinct pathogenic mechanisms and outcomes.
  雑誌
Nat Med 24:679-690 (2018)
DOI:10.1038/s41591-018-0016-8
文献    
  著者
Pasqualucci L, Dalla-Favera R
  タイトル
Genetics of diffuse large B-cell lymphoma.
  雑誌
Blood 131:2307-2319 (2018)
DOI:10.1182/blood-2017-11-764332
文献    
  著者
Lohr JG, Stojanov P, Lawrence MS, Auclair D, Chapuy B, Sougnez C, Cruz-Gordillo P, Knoechel B, Asmann YW, Slager SL, Novak AJ, Dogan A, Ansell SM, Link BK, Zou L, Gould J, Saksena G, Stransky N, Rangel-Escareno C, Fernandez-Lopez JC, Hidalgo-Miranda A, Melendez-Zajgla J, Hernandez-Lemus E, Schwarz-Cruz y Celis A, Imaz-Rosshandler I, Ojesina AI, Jung J, Pedamallu CS, Lander ES, Habermann TM, Cerhan JR, Shipp MA, Getz G, Golub TR
  タイトル
Discovery and prioritization of somatic mutations in diffuse large B-cell lymphoma (DLBCL) by whole-exome sequencing.
  雑誌
Proc Natl Acad Sci U S A 109:3879-84 (2012)
DOI:10.1073/pnas.1121343109
文献    
  著者
Coccaro N, Anelli L, Zagaria A, Perrone T, Specchia G, Albano F
  タイトル
Molecular Complexity of Diffuse Large B-Cell Lymphoma: Can It Be a Roadmap for Precision Medicine?
  雑誌
Cancers (Basel) 12:E185 (2020)
DOI:10.3390/cancers12010185
LinkDB    

» English version

DBGET integrated database retrieval system