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Diffuse Large B-Cell Lymphoma Unclassifiable

MedGen UID:
318287
•Concept ID:
C1710498
•
Neoplastic Process
Synonyms: Diffuse Large B-Cell Lymphoma Indeterminate; DLBCL Unclassifiable; Type 3 Diffuse Large B-Cell Lymphoma

Definition

A biologic subset of diffuse large B-cell lymphomas (DLBCL) that do not overexpress the genes whose overexpression characterizes the germinal center B-cell-like and activated B-cell-like DLBCL. Morphologically, these lymphomas are either immunoblastic or centroblastic. Patients with this type of diffuse large B-cell lymphoma are reported to have a less favorable outcome, similar to those with an activated B-cell-like gene expression profile. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDiffuse Large B-Cell Lymphoma Unclassifiable

Professional guidelines

PubMed

Advances in biology, diagnosis and treatment of DLBCL.
Shi Y, Xu Y, Shen H, Jin J, Tong H, Xie W
Ann Hematol 2024 Sep;103(9):3315-3334. Epub 2024 Jul 17 doi: 10.1007/s00277-024-05880-z. PMID: 39017945Free PMC Article
Diffuse large B-cell lymphoma: 2019 update on diagnosis, risk stratification, and treatment.
Liu Y, Barta SK
Am J Hematol 2019 May;94(5):604-616. doi: 10.1002/ajh.25460. PMID: 30859597
The importance of radiotherapy on diffuse large B cell lymphoma treatment: a current review.
Mendes F, Domingues C, Teixo R, Abrantes AM, Gonçalves AC, Nobre-Gois I, Jacobetty M, Sarmento AB, Botelho MF, Rosa MS
Cancer Metastasis Rev 2015 Sep;34(3):511-25. doi: 10.1007/s10555-015-9581-1. PMID: 26267803

Recent clinical studies

Etiology

Targeted sequencing in DLBCL, molecular subtypes, and outcomes: a Haematological Malignancy Research Network report.
Lacy SE, Barrans SL, Beer PA, Painter D, Smith AG, Roman E, Cooke SL, Ruiz C, Glover P, Van Hoppe SJL, Webster N, Campbell PJ, Tooze RM, Patmore R, Burton C, Crouch S, Hodson DJ
Blood 2020 May 14;135(20):1759-1771. doi: 10.1182/blood.2019003535. PMID: 32187361Free PMC Article
Diffuse large B-cell lymphoma: 2019 update on diagnosis, risk stratification, and treatment.
Liu Y, Barta SK
Am J Hematol 2019 May;94(5):604-616. doi: 10.1002/ajh.25460. PMID: 30859597
Diffuse large B-cell lymphoma.
Li S, Young KH, Medeiros LJ
Pathology 2018 Jan;50(1):74-87. Epub 2017 Nov 20 doi: 10.1016/j.pathol.2017.09.006. PMID: 29167021
High grade B-cell lymphoma with rearrangements of MYC and BCL2 and/or BCL6: Double hit and triple hit lymphomas and double expressing lymphoma.
Rosenthal A, Younes A
Blood Rev 2017 Mar;31(2):37-42. Epub 2016 Sep 30 doi: 10.1016/j.blre.2016.09.004. PMID: 27717585Free PMC Article
Diagnosis of 'double hit' diffuse large B-cell lymphoma and B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt lymphoma: when and how, FISH versus IHC.
Swerdlow SH
Hematology Am Soc Hematol Educ Program 2014 Dec 5;2014(1):90-9. Epub 2014 Nov 18 doi: 10.1182/asheducation-2014.1.90. PMID: 25696840

Diagnosis

CD5 Gene Signature Identifies Diffuse Large B-Cell Lymphomas Sensitive to Bruton's Tyrosine Kinase Inhibition.
Cooper A, Tumuluru S, Kissick K, Venkataraman G, Song JY, Lytle A, Duns G, Yu J, Kotlov N, Bagaev A, Hodkinson B, Srinivasan S, Smith SM, Scott DW, Steidl C, Godfrey JK, Kline J
J Clin Oncol 2024 Feb 1;42(4):467-480. Epub 2023 Dec 11 doi: 10.1200/JCO.23.01574. PMID: 38079587
Targeted sequencing in DLBCL, molecular subtypes, and outcomes: a Haematological Malignancy Research Network report.
Lacy SE, Barrans SL, Beer PA, Painter D, Smith AG, Roman E, Cooke SL, Ruiz C, Glover P, Van Hoppe SJL, Webster N, Campbell PJ, Tooze RM, Patmore R, Burton C, Crouch S, Hodson DJ
Blood 2020 May 14;135(20):1759-1771. doi: 10.1182/blood.2019003535. PMID: 32187361Free PMC Article
Diffuse large B-cell lymphoma: 2019 update on diagnosis, risk stratification, and treatment.
Liu Y, Barta SK
Am J Hematol 2019 May;94(5):604-616. doi: 10.1002/ajh.25460. PMID: 30859597
Diffuse large B-cell lymphoma.
Li S, Young KH, Medeiros LJ
Pathology 2018 Jan;50(1):74-87. Epub 2017 Nov 20 doi: 10.1016/j.pathol.2017.09.006. PMID: 29167021
Double-hit B-cell lymphomas.
Aukema SM, Siebert R, Schuuring E, van Imhoff GW, Kluin-Nelemans HC, Boerma EJ, Kluin PM
Blood 2011 Feb 24;117(8):2319-31. Epub 2010 Nov 30 doi: 10.1182/blood-2010-09-297879. PMID: 21119107

Therapy

CD5 Gene Signature Identifies Diffuse Large B-Cell Lymphomas Sensitive to Bruton's Tyrosine Kinase Inhibition.
Cooper A, Tumuluru S, Kissick K, Venkataraman G, Song JY, Lytle A, Duns G, Yu J, Kotlov N, Bagaev A, Hodkinson B, Srinivasan S, Smith SM, Scott DW, Steidl C, Godfrey JK, Kline J
J Clin Oncol 2024 Feb 1;42(4):467-480. Epub 2023 Dec 11 doi: 10.1200/JCO.23.01574. PMID: 38079587
Diffuse Large B Cell Lymphoma Arising in Patients with Preexisting Hodgkin Lymphoma.
Bellitti E, Masciopinto P, Musto P, Arcuti E, Mastracci L, Opinto G, Ciavarella S, Guarini A, Cazzato G, Specchia G, Maiorano E, Gaudio F, Ingravallo G
Curr Oncol 2022 Aug 25;29(9):6115-6124. doi: 10.3390/curroncol29090480. PMID: 36135049Free PMC Article
Genetics and Pathogenesis of Diffuse Large B-Cell Lymphoma.
Schmitz R, Wright GW, Huang DW, Johnson CA, Phelan JD, Wang JQ, Roulland S, Kasbekar M, Young RM, Shaffer AL, Hodson DJ, Xiao W, Yu X, Yang Y, Zhao H, Xu W, Liu X, Zhou B, Du W, Chan WC, Jaffe ES, Gascoyne RD, Connors JM, Campo E, Lopez-Guillermo A, Rosenwald A, Ott G, Delabie J, Rimsza LM, Tay Kuang Wei K, Zelenetz AD, Leonard JP, Bartlett NL, Tran B, Shetty J, Zhao Y, Soppet DR, Pittaluga S, Wilson WH, Staudt LM
N Engl J Med 2018 Apr 12;378(15):1396-1407. doi: 10.1056/NEJMoa1801445. PMID: 29641966Free PMC Article
High grade B-cell lymphoma with rearrangements of MYC and BCL2 and/or BCL6: Double hit and triple hit lymphomas and double expressing lymphoma.
Rosenthal A, Younes A
Blood Rev 2017 Mar;31(2):37-42. Epub 2016 Sep 30 doi: 10.1016/j.blre.2016.09.004. PMID: 27717585Free PMC Article
Burkitt lymphoma in adults.
Linch DC
Br J Haematol 2012 Mar;156(6):693-703. Epub 2011 Sep 19 doi: 10.1111/j.1365-2141.2011.08877.x. PMID: 21923642

Prognosis

Targeted sequencing in DLBCL, molecular subtypes, and outcomes: a Haematological Malignancy Research Network report.
Lacy SE, Barrans SL, Beer PA, Painter D, Smith AG, Roman E, Cooke SL, Ruiz C, Glover P, Van Hoppe SJL, Webster N, Campbell PJ, Tooze RM, Patmore R, Burton C, Crouch S, Hodson DJ
Blood 2020 May 14;135(20):1759-1771. doi: 10.1182/blood.2019003535. PMID: 32187361Free PMC Article
Diffuse large B-cell lymphoma: 2019 update on diagnosis, risk stratification, and treatment.
Liu Y, Barta SK
Am J Hematol 2019 May;94(5):604-616. doi: 10.1002/ajh.25460. PMID: 30859597
Genetics and Pathogenesis of Diffuse Large B-Cell Lymphoma.
Schmitz R, Wright GW, Huang DW, Johnson CA, Phelan JD, Wang JQ, Roulland S, Kasbekar M, Young RM, Shaffer AL, Hodson DJ, Xiao W, Yu X, Yang Y, Zhao H, Xu W, Liu X, Zhou B, Du W, Chan WC, Jaffe ES, Gascoyne RD, Connors JM, Campo E, Lopez-Guillermo A, Rosenwald A, Ott G, Delabie J, Rimsza LM, Tay Kuang Wei K, Zelenetz AD, Leonard JP, Bartlett NL, Tran B, Shetty J, Zhao Y, Soppet DR, Pittaluga S, Wilson WH, Staudt LM
N Engl J Med 2018 Apr 12;378(15):1396-1407. doi: 10.1056/NEJMoa1801445. PMID: 29641966Free PMC Article
Diffuse large B-cell lymphoma.
Li S, Young KH, Medeiros LJ
Pathology 2018 Jan;50(1):74-87. Epub 2017 Nov 20 doi: 10.1016/j.pathol.2017.09.006. PMID: 29167021
High grade B-cell lymphoma with rearrangements of MYC and BCL2 and/or BCL6: Double hit and triple hit lymphomas and double expressing lymphoma.
Rosenthal A, Younes A
Blood Rev 2017 Mar;31(2):37-42. Epub 2016 Sep 30 doi: 10.1016/j.blre.2016.09.004. PMID: 27717585Free PMC Article

Clinical prediction guides

Genetic and transcriptomic analyses of diffuse large B-cell lymphoma patients with poor outcomes within two years of diagnosis.
Ren W, Wan H, Own SA, Berglund M, Wang X, Yang M, Li X, Liu D, Ye X, Sonnevi K, Enblad G, Amini RM, Sander B, Wu K, Zhang H, Wahlin BE, Smedby KE, Pan-Hammarström Q
Leukemia 2024 Mar;38(3):610-620. Epub 2023 Dec 29 doi: 10.1038/s41375-023-02120-7. PMID: 38158444Free PMC Article
CD5 Gene Signature Identifies Diffuse Large B-Cell Lymphomas Sensitive to Bruton's Tyrosine Kinase Inhibition.
Cooper A, Tumuluru S, Kissick K, Venkataraman G, Song JY, Lytle A, Duns G, Yu J, Kotlov N, Bagaev A, Hodkinson B, Srinivasan S, Smith SM, Scott DW, Steidl C, Godfrey JK, Kline J
J Clin Oncol 2024 Feb 1;42(4):467-480. Epub 2023 Dec 11 doi: 10.1200/JCO.23.01574. PMID: 38079587
APR-246 triggers ferritinophagy and ferroptosis of diffuse large B-cell lymphoma cells with distinct TP53 mutations.
Hong Y, Ren T, Wang X, Liu X, Fei Y, Meng S, Han X, Sun C, Shen H, Li L, Qiu L, Qian Z, Zhou S, Zhang H, Wang X
Leukemia 2022 Sep;36(9):2269-2280. Epub 2022 Jul 14 doi: 10.1038/s41375-022-01634-w. PMID: 35835991
Diffuse large B-cell lymphoma.
Li S, Young KH, Medeiros LJ
Pathology 2018 Jan;50(1):74-87. Epub 2017 Nov 20 doi: 10.1016/j.pathol.2017.09.006. PMID: 29167021
Double-hit B-cell lymphomas.
Aukema SM, Siebert R, Schuuring E, van Imhoff GW, Kluin-Nelemans HC, Boerma EJ, Kluin PM
Blood 2011 Feb 24;117(8):2319-31. Epub 2010 Nov 30 doi: 10.1182/blood-2010-09-297879. PMID: 21119107

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