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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

Guideline Concordance of Treatment and Outcomes Among Adult Non-Hodgkin Lymphoma Patients in Sub-Saharan Africa: A Multinational, Population-Based Cohort.
Mezger NCS, Hämmerl L, Griesel M, Seraphin TP, Joko-Fru YW, Feuchtner J, Zietsman A, Péko JF, Tadesse F, Buziba NG, Wabinga H, Nyanchama M, Chokunonga E, Kéita M, N'da G, Lorenzoni CF, Akele-Akpo MT, Mezger JM, Binder M, Liu B, Bauer M, Henke O, Jemal A, Kantelhardt EJ
Oncologist 2023 Nov 2;28(11):e1017-e1030. doi: 10.1093/oncolo/oyad157. PMID: 37368350Free 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

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
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

Unclassifiable lymphoma in pregnancy.
Hernández Martínez M, Lizán Tudela C, Saus Carreres A
BMJ Case Rep 2021 Feb 24;14(2) doi: 10.1136/bcr-2020-239462. PMID: 33627347Free PMC Article
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

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
Unclassifiable lymphoma in pregnancy.
Hernández Martínez M, Lizán Tudela C, Saus Carreres A
BMJ Case Rep 2021 Feb 24;14(2) doi: 10.1136/bcr-2020-239462. PMID: 33627347Free PMC Article
Molecular Subtyping of Diffuse Large B-Cell Lymphoma Using a Novel Quantitative RT-PCR Assay.
Ta R, Santini C, Gou P, Lee G, Tai YC, O'Brien C, Fontecha M, Grant C, Bacon L, Finn S, Vandenberghe E, Quinn F, Dua R, Flavin R
J Mol Diagn 2021 Mar;23(3):323-340. Epub 2020 Dec 29 doi: 10.1016/j.jmoldx.2020.11.013. PMID: 33385586
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

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

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
MYC/BCL2 double-hit high-grade B-cell lymphoma.
Li S, Lin P, Young KH, Kanagal-Shamanna R, Yin CC, Medeiros LJ
Adv Anat Pathol 2013 Sep;20(5):315-26. doi: 10.1097/PAP.0b013e3182a289f2. PMID: 23939148
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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