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Diffuse large B-cell lymphoma activated B-cell type

MedGen UID:
272545
•Concept ID:
C1333296
•
Neoplastic Process
Synonyms: Activated B-Cell-Like Diffuse Large B-Cell Lymphoma; Diffuse Large B-Cell Lymphoma Activated B-Cell Type; diffuse large B-cell lymphoma activated B-cell type; Diffuse Large B-Cell Lymphoma Non-GC/ABC; Diffuse Large B-Cell Lymphoma Non-Germinal Center/Activated B-Cell Type; Diffuse Large B-Cell Lymphoma with an Activated B-Cell Expression Profile; DLBCL Activated B-Cell Type; Non-GC/ABC DLBCL
 
Monarch Initiative: MONDO:0850418

Definition

A biologic subset of diffuse large B-cell lymphomas with a unique molecular signature or expression profile. It represents approximately 30% of diffuse large B-cell lymphomas, and is characterized by the expression of CD44, PKCbeta1, Cyclin D2, BCL-2, and IRF4/MUM1 genes. Morphologically, these lymphomas are either centroblastic or immunoblastic (ratio 2:1). Patients with this type of diffuse large B-cell lymphoma are reported to have a less favorable outcome compared to those with a germinal center B-cell expression profile, with a 5-year survival rate of 35% and a median survival of 2 years. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDiffuse large B-cell lymphoma activated B-cell type

Professional guidelines

PubMed

2021 Update on Diffuse large B cell lymphoma: A review of current data and potential applications on risk stratification and management.
Susanibar-Adaniya S, Barta SK
Am J Hematol 2021 May 1;96(5):617-629. Epub 2021 Mar 19 doi: 10.1002/ajh.26151. PMID: 33661537Free 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
Advances in Diagnosis and Management of Diffuse Large B-cell Lymphoma.
Cabanillas F, Shah B
Clin Lymphoma Myeloma Leuk 2017 Dec;17(12):783-796. Epub 2017 Nov 7 doi: 10.1016/j.clml.2017.10.007. PMID: 29126866

Recent clinical studies

Etiology

Whole-genome landscape of adult T-cell leukemia/lymphoma.
Kogure Y, Kameda T, Koya J, Yoshimitsu M, Nosaka K, Yasunaga JI, Imaizumi Y, Watanabe M, Saito Y, Ito Y, McClure MB, Tabata M, Shingaki S, Yoshifuji K, Chiba K, Okada A, Kakiuchi N, Nannya Y, Kamiunten A, Tahira Y, Akizuki K, Sekine M, Shide K, Hidaka T, Kubuki Y, Kitanaka A, Hidaka M, Nakano N, Utsunomiya A, Sica RA, Acuna-Villaorduna A, Janakiram M, Shah U, Ramos JC, Shibata T, Takeuchi K, Takaori-Kondo A, Miyazaki Y, Matsuoka M, Ishitsuka K, Shiraishi Y, Miyano S, Ogawa S, Ye BH, Shimoda K, Kataoka K
Blood 2022 Feb 17;139(7):967-982. doi: 10.1182/blood.2021013568. PMID: 34695199Free PMC Article
2021 Update on Diffuse large B cell lymphoma: A review of current data and potential applications on risk stratification and management.
Susanibar-Adaniya S, Barta SK
Am J Hematol 2021 May 1;96(5):617-629. Epub 2021 Mar 19 doi: 10.1002/ajh.26151. PMID: 33661537Free 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
Confirmation of the molecular classification of diffuse large B-cell lymphoma by immunohistochemistry using a tissue microarray.
Hans CP, Weisenburger DD, Greiner TC, Gascoyne RD, Delabie J, Ott G, Müller-Hermelink HK, Campo E, Braziel RM, Jaffe ES, Pan Z, Farinha P, Smith LM, Falini B, Banham AH, Rosenwald A, Staudt LM, Connors JM, Armitage JO, Chan WC
Blood 2004 Jan 1;103(1):275-82. Epub 2003 Sep 22 doi: 10.1182/blood-2003-05-1545. PMID: 14504078

Diagnosis

CREBBP/EP300 mutations promoted tumor progression in diffuse large B-cell lymphoma through altering tumor-associated macrophage polarization via FBXW7-NOTCH-CCL2/CSF1 axis.
Huang YH, Cai K, Xu PP, Wang L, Huang CX, Fang Y, Cheng S, Sun XJ, Liu F, Huang JY, Ji MM, Zhao WL
Signal Transduct Target Ther 2021 Jan 11;6(1):10. doi: 10.1038/s41392-020-00437-8. PMID: 33431788Free 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
Confirmation of the molecular classification of diffuse large B-cell lymphoma by immunohistochemistry using a tissue microarray.
Hans CP, Weisenburger DD, Greiner TC, Gascoyne RD, Delabie J, Ott G, Müller-Hermelink HK, Campo E, Braziel RM, Jaffe ES, Pan Z, Farinha P, Smith LM, Falini B, Banham AH, Rosenwald A, Staudt LM, Connors JM, Armitage JO, Chan WC
Blood 2004 Jan 1;103(1):275-82. Epub 2003 Sep 22 doi: 10.1182/blood-2003-05-1545. PMID: 14504078
Distinct types of diffuse large B-cell lymphoma identified by gene expression profiling.
Alizadeh AA, Eisen MB, Davis RE, Ma C, Lossos IS, Rosenwald A, Boldrick JC, Sabet H, Tran T, Yu X, Powell JI, Yang L, Marti GE, Moore T, Hudson J Jr, Lu L, Lewis DB, Tibshirani R, Sherlock G, Chan WC, Greiner TC, Weisenburger DD, Armitage JO, Warnke R, Levy R, Wilson W, Grever MR, Byrd JC, Botstein D, Brown PO, Staudt LM
Nature 2000 Feb 3;403(6769):503-11. doi: 10.1038/35000501. PMID: 10676951

Therapy

2021 Update on Diffuse large B cell lymphoma: A review of current data and potential applications on risk stratification and management.
Susanibar-Adaniya S, Barta SK
Am J Hematol 2021 May 1;96(5):617-629. Epub 2021 Mar 19 doi: 10.1002/ajh.26151. PMID: 33661537Free PMC Article
ROBUST: A Phase III Study of Lenalidomide Plus R-CHOP Versus Placebo Plus R-CHOP in Previously Untreated Patients With ABC-Type Diffuse Large B-Cell Lymphoma.
Nowakowski GS, Chiappella A, Gascoyne RD, Scott DW, Zhang Q, Jurczak W, Özcan M, Hong X, Zhu J, Jin J, Belada D, Bergua JM, Piazza F, Mócikova H, Molinari AL, Yoon DH, Cavallo F, Tani M, Yamamoto K, Izutsu K, Kato K, Czuczman M, Hersey S, Kilcoyne A, Russo J, Hudak K, Zhang J, Wade S, Witzig TE, Vitolo U
J Clin Oncol 2021 Apr 20;39(12):1317-1328. Epub 2021 Feb 23 doi: 10.1200/JCO.20.01366. PMID: 33621109Free PMC Article
A randomized, open-label, Phase III study of obinutuzumab or rituximab plus CHOP in patients with previously untreated diffuse large B-Cell lymphoma: final analysis of GOYA.
Sehn LH, Martelli M, Trněný M, Liu W, Bolen CR, Knapp A, Sahin D, Sellam G, Vitolo U
J Hematol Oncol 2020 Jun 6;13(1):71. doi: 10.1186/s13045-020-00900-7. PMID: 32505213Free PMC Article
Precision medicine for human cancers with Notch signaling dysregulation (Review).
Katoh M, Katoh M
Int J Mol Med 2020 Feb;45(2):279-297. Epub 2019 Dec 4 doi: 10.3892/ijmm.2019.4418. PMID: 31894255Free PMC Article
The cancer driver genes IDH1/2, JARID1C/ KDM5C, and UTX/ KDM6A: crosstalk between histone demethylation and hypoxic reprogramming in cancer metabolism.
Chang S, Yim S, Park H
Exp Mol Med 2019 Jun 20;51(6):1-17. doi: 10.1038/s12276-019-0230-6. PMID: 31221981Free PMC Article

Prognosis

Whole-genome landscape of adult T-cell leukemia/lymphoma.
Kogure Y, Kameda T, Koya J, Yoshimitsu M, Nosaka K, Yasunaga JI, Imaizumi Y, Watanabe M, Saito Y, Ito Y, McClure MB, Tabata M, Shingaki S, Yoshifuji K, Chiba K, Okada A, Kakiuchi N, Nannya Y, Kamiunten A, Tahira Y, Akizuki K, Sekine M, Shide K, Hidaka T, Kubuki Y, Kitanaka A, Hidaka M, Nakano N, Utsunomiya A, Sica RA, Acuna-Villaorduna A, Janakiram M, Shah U, Ramos JC, Shibata T, Takeuchi K, Takaori-Kondo A, Miyazaki Y, Matsuoka M, Ishitsuka K, Shiraishi Y, Miyano S, Ogawa S, Ye BH, Shimoda K, Kataoka K
Blood 2022 Feb 17;139(7):967-982. doi: 10.1182/blood.2021013568. PMID: 34695199Free PMC Article
Immunogenomic Landscape of Hematological Malignancies.
Dufva O, Pölönen P, Brück O, Keränen MAI, Klievink J, Mehtonen J, Huuhtanen J, Kumar A, Malani D, Siitonen S, Kankainen M, Ghimire B, Lahtela J, Mattila P, Vähä-Koskela M, Wennerberg K, Granberg K, Leivonen SK, Meriranta L, Heckman C, Leppä S, Nykter M, Lohi O, Heinäniemi M, Mustjoki S
Cancer Cell 2020 Sep 14;38(3):380-399.e13. Epub 2020 Jul 9 doi: 10.1016/j.ccell.2020.06.002. PMID: 32649887
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
Confirmation of the molecular classification of diffuse large B-cell lymphoma by immunohistochemistry using a tissue microarray.
Hans CP, Weisenburger DD, Greiner TC, Gascoyne RD, Delabie J, Ott G, Müller-Hermelink HK, Campo E, Braziel RM, Jaffe ES, Pan Z, Farinha P, Smith LM, Falini B, Banham AH, Rosenwald A, Staudt LM, Connors JM, Armitage JO, Chan WC
Blood 2004 Jan 1;103(1):275-82. Epub 2003 Sep 22 doi: 10.1182/blood-2003-05-1545. PMID: 14504078

Clinical prediction guides

ROBUST: A Phase III Study of Lenalidomide Plus R-CHOP Versus Placebo Plus R-CHOP in Previously Untreated Patients With ABC-Type Diffuse Large B-Cell Lymphoma.
Nowakowski GS, Chiappella A, Gascoyne RD, Scott DW, Zhang Q, Jurczak W, Özcan M, Hong X, Zhu J, Jin J, Belada D, Bergua JM, Piazza F, Mócikova H, Molinari AL, Yoon DH, Cavallo F, Tani M, Yamamoto K, Izutsu K, Kato K, Czuczman M, Hersey S, Kilcoyne A, Russo J, Hudak K, Zhang J, Wade S, Witzig TE, Vitolo U
J Clin Oncol 2021 Apr 20;39(12):1317-1328. Epub 2021 Feb 23 doi: 10.1200/JCO.20.01366. PMID: 33621109Free PMC Article
CREBBP/EP300 mutations promoted tumor progression in diffuse large B-cell lymphoma through altering tumor-associated macrophage polarization via FBXW7-NOTCH-CCL2/CSF1 axis.
Huang YH, Cai K, Xu PP, Wang L, Huang CX, Fang Y, Cheng S, Sun XJ, Liu F, Huang JY, Ji MM, Zhao WL
Signal Transduct Target Ther 2021 Jan 11;6(1):10. doi: 10.1038/s41392-020-00437-8. PMID: 33431788Free PMC Article
Immunogenomic Landscape of Hematological Malignancies.
Dufva O, Pölönen P, Brück O, Keränen MAI, Klievink J, Mehtonen J, Huuhtanen J, Kumar A, Malani D, Siitonen S, Kankainen M, Ghimire B, Lahtela J, Mattila P, Vähä-Koskela M, Wennerberg K, Granberg K, Leivonen SK, Meriranta L, Heckman C, Leppä S, Nykter M, Lohi O, Heinäniemi M, Mustjoki S
Cancer Cell 2020 Sep 14;38(3):380-399.e13. Epub 2020 Jul 9 doi: 10.1016/j.ccell.2020.06.002. PMID: 32649887
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
Confirmation of the molecular classification of diffuse large B-cell lymphoma by immunohistochemistry using a tissue microarray.
Hans CP, Weisenburger DD, Greiner TC, Gascoyne RD, Delabie J, Ott G, Müller-Hermelink HK, Campo E, Braziel RM, Jaffe ES, Pan Z, Farinha P, Smith LM, Falini B, Banham AH, Rosenwald A, Staudt LM, Connors JM, Armitage JO, Chan WC
Blood 2004 Jan 1;103(1):275-82. Epub 2003 Sep 22 doi: 10.1182/blood-2003-05-1545. PMID: 14504078

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