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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

Professional guidelines

PubMed

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
Liu Y, Barta SK
Am J Hematol 2019 May;94(5):604-616. doi: 10.1002/ajh.25460. PMID: 30859597
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

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
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
Liu Y, Barta SK
Am J Hematol 2019 May;94(5):604-616. doi: 10.1002/ajh.25460. PMID: 30859597
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
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

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
Liu Y, Barta SK
Am J Hematol 2019 May;94(5):604-616. doi: 10.1002/ajh.25460. PMID: 30859597
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
Elton TS, Selemon H, Elton SM, Parinandi NL
Gene 2013 Dec 10;532(1):1-12. Epub 2012 Dec 14 doi: 10.1016/j.gene.2012.12.009. PMID: 23246696
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

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
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
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
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
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

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
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
Liu Y, Barta SK
Am J Hematol 2019 May;94(5):604-616. doi: 10.1002/ajh.25460. PMID: 30859597
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
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

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
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
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
Ngo VN, Young RM, Schmitz R, Jhavar S, Xiao W, Lim KH, Kohlhammer H, Xu W, Yang Y, Zhao H, Shaffer AL, Romesser P, Wright G, Powell J, Rosenwald A, Muller-Hermelink HK, Ott G, Gascoyne RD, Connors JM, Rimsza LM, Campo E, Jaffe ES, Delabie J, Smeland EB, Fisher RI, Braziel RM, Tubbs RR, Cook JR, Weisenburger DD, Chan WC, Staudt LM
Nature 2011 Feb 3;470(7332):115-9. Epub 2010 Dec 22 doi: 10.1038/nature09671. PMID: 21179087Free PMC Article
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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