KEGG   DISEASE: Diffuse large B-cell lymphoma, not otherwise specified
H02434                      Disease                                
Diffuse large B-cell lymphoma, not otherwise specified
Non-Hodgkin lymphoma [DS:H02418]
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).
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
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]
Rituximab and hyaluronidase [DR:D11393]
Pembrolizumab [DR:D10574]
Tafasitamab [DR:D11601] (CD19-directed)
Polatuzumab vedotin [DR:D10761]
Loncastuximab tesirine [DR:D11338]
Epcoritamab [DR:D12368]
Glofitamab [DR:D11833]
Lisocabtagene maraleucel [DR:D11990]
Other DBs
ICD-11: 2A81.Z
ICD-10: C83.3
MeSH: D016403
Li S, Young KH, Medeiros LJ
Diffuse large B-cell lymphoma.
Pathology 50:74-87 (2018)
Chiappella A, Crombie J, Guidetti A, Vitolo U, Armand P, Corradini P
Are We Ready to Treat Diffuse Large B-cell and High-Grade Lymphoma According to Major Genetic Subtypes?
Hemasphere 3:e284 (2019)
Ennishi D, Hsi ED, Steidl C, Scott DW
Toward a New Molecular Taxonomy of Diffuse Large B-cell Lymphoma.
Cancer Discov 2159-8290.CD-20-0174 (2020)
Ohmachi K
JSH practical guidelines for hematological malignancies, 2018: II. Lymphoma-5-diffuse large B-cell lymphoma, not otherwise specified (DLBCL, NOS).
Int J Hematol 110:131-146 (2019)
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
Molecular subtypes of diffuse large B cell lymphoma are associated with distinct pathogenic mechanisms and outcomes.
Nat Med 24:679-690 (2018)
Pasqualucci L, Dalla-Favera R
Genetics of diffuse large B-cell lymphoma.
Blood 131:2307-2319 (2018)
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
Discovery and prioritization of somatic mutations in diffuse large B-cell lymphoma (DLBCL) by whole-exome sequencing.
Proc Natl Acad Sci U S A 109:3879-84 (2012)
Coccaro N, Anelli L, Zagaria A, Perrone T, Specchia G, Albano F
Molecular Complexity of Diffuse Large B-Cell Lymphoma: Can It Be a Roadmap for Precision Medicine?
Cancers (Basel) 12:E185 (2020)

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