KEGG   DISEASE: Diffuse large B-cell lymphoma, not otherwise specified
Entry
H02434                      Disease                                

Name
Diffuse large B-cell lymphoma, not otherwise specified
  Supergrp
Non-Hodgkin lymphoma [DS:H02418]
Description
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).
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of haematopoietic and lymphoid tissues
   H02434  Diffuse large B-cell lymphoma, not otherwise specified
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Mature B-cell neoplasms
    2A81  Diffuse large B-cell lymphomas
     H02434  Diffuse large B-cell lymphoma, not otherwise specified
Pathway
hsa04151  PI3K-Akt signaling pathway
hsa04210  Apoptosis
hsa04662  B cell receptor signaling pathway
hsa04064  NF-kappa B signaling pathway
Gene
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]
Drug
Tafasitamab [DR:D11601] (CD19-directed)
Comment
ICD-O-3: M9680/3 Malignant lymphoma, large cell, diffuse NOS
Other DBs
ICD-11: 2A81.Z
ICD-10: C83.3
MeSH: D016403
Reference
  Authors
Li S, Young KH, Medeiros LJ
  Title
Diffuse large B-cell lymphoma.
  Journal
Pathology 50:74-87 (2018)
DOI:10.1016/j.pathol.2017.09.006
Reference
  Authors
Chiappella A, Crombie J, Guidetti A, Vitolo U, Armand P, Corradini P
  Title
Are We Ready to Treat Diffuse Large B-cell and High-Grade Lymphoma According to Major Genetic Subtypes?
  Journal
Hemasphere 3:e284 (2019)
DOI:10.1097/HS9.0000000000000284
Reference
  Authors
Ennishi D, Hsi ED, Steidl C, Scott DW
  Title
Toward a New Molecular Taxonomy of Diffuse Large B-cell Lymphoma.
  Journal
Cancer Discov 2159-8290.CD-20-0174 (2020)
DOI:10.1158/2159-8290.CD-20-0174
Reference
  Authors
Ohmachi K
  Title
JSH practical guidelines for hematological malignancies, 2018: II. Lymphoma-5-diffuse large B-cell lymphoma, not otherwise specified (DLBCL, NOS).
  Journal
Int J Hematol 110:131-146 (2019)
DOI:10.1007/s12185-019-02681-3
Reference
  Authors
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
  Title
Molecular subtypes of diffuse large B cell lymphoma are associated with distinct pathogenic mechanisms and outcomes.
  Journal
Nat Med 24:679-690 (2018)
DOI:10.1038/s41591-018-0016-8
Reference
  Authors
Pasqualucci L, Dalla-Favera R
  Title
Genetics of diffuse large B-cell lymphoma.
  Journal
Blood 131:2307-2319 (2018)
DOI:10.1182/blood-2017-11-764332
Reference
  Authors
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
  Title
Discovery and prioritization of somatic mutations in diffuse large B-cell lymphoma (DLBCL) by whole-exome sequencing.
  Journal
Proc Natl Acad Sci U S A 109:3879-84 (2012)
DOI:10.1073/pnas.1121343109
Reference
  Authors
Coccaro N, Anelli L, Zagaria A, Perrone T, Specchia G, Albano F
  Title
Molecular Complexity of Diffuse Large B-Cell Lymphoma: Can It Be a Roadmap for Precision Medicine?
  Journal
Cancers (Basel) 12:E185 (2020)
DOI:10.3390/cancers12010185
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