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High Grade B-Cell Lymphoma, Not Otherwise Specified

MedGen UID:
395909
•Concept ID:
C2698294
•
Neoplastic Process
Synonyms: B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma; High Grade B-Cell Lymphoma, NOS; High-Grade B-Cell Lymphoma, NOS; High-Grade B-Cell Lymphoma, Not Otherwise Specified
SNOMED CT: B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma (12341000132100)

Definition

High-grade B-cell lymphoma with blastoid features or features between diffuse large B-cell lymphoma and Burkitt lymphoma that lacks MYC, BCL2, and BCL6 gene rearrangements. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHigh Grade B-Cell Lymphoma, Not Otherwise Specified

Professional guidelines

PubMed

Favourable outcomes of double-hit/double-expressor lymphoma and high-grade B-cell lymphoma, not otherwise specified after early dose-intensive treatment and up-front autologous stem cell transplantation: a single-centre retrospective experience.
Strüßmann T, Glatzki F, Engelhardt M, Mertelsmann R, Duyster J, Finke J, Marks R
Br J Haematol 2022 Aug;198(4):776-779. Epub 2022 May 27 doi: 10.1111/bjh.18280. PMID: 35620830
The "Burkitt-like" immunophenotype and genotype is rarely encountered in diffuse large B cell lymphoma and high-grade B cell lymphoma, NOS.
Hüttl KS, Staiger AM, Richter J, Ott MM, Kalmbach S, Klapper W, Biesdorf AS, Trümper L, Rosenwald A, Ziepert M, Horn H, Ott G
Virchows Arch 2021 Sep;479(3):575-583. Epub 2021 Mar 2 doi: 10.1007/s00428-021-03050-4. PMID: 33655392

Recent clinical studies

Etiology

High-grade B-cell lymphoma, not otherwise specified: CNS involvement and outcomes in a multi-institutional series.
Epperla N, Zayac AS, Landsburg DJ, Bock AM, Nowakowski GS, Ayers EC, Girton M, Hu M, Beckman A, Li S, Medeiros LJ, Chang JE, Kurt H, Sandoval-Sus J, Ansari-Lari MA, Kothari SK, Kress A, Xu ML, Torka P, Sundaram S, Smith SD, Naresh KN, Karimi Y, Bond DA, Evens AM, Naik SG, Kamdar M, Haverkos BM, Karmali R, Farooq U, Vose JM, Rubinstein P, Chaudhry A, Olszewski AJ
Blood Adv 2024 Oct 22;8(20):5355-5364. doi: 10.1182/bloodadvances.2024013791. PMID: 39189932
The Clinicopathologic Features and Molecular Signatures of Blastoid High-Grade B Cell Lymphoma, Not Otherwise Specified.
Qiu L, Lin P, Khanlari M, Xu J, Cohen EN, Garces S, Miranda RN, Wang W, Fang H, Bueso-Ramos CE, Medeiros LJ, Li S
Mod Pathol 2023 Dec;36(12):100349. Epub 2023 Oct 10 doi: 10.1016/j.modpat.2023.100349. PMID: 37820764
High-grade B-cell lymphoma, not otherwise specified: a multi-institutional retrospective study.
Zayac AS, Landsburg DJ, Hughes ME, Bock AM, Nowakowski GS, Ayers EC, Girton M, Hu M, Beckman AK, Li S, Medeiros LJ, Chang JE, Stepanovic A, Kurt H, Sandoval-Sus J, Ansari-Lari MA, Kothari SK, Kress A, Xu ML, Torka P, Sundaram S, Smith SD, Naresh KN, Karimi YH, Epperla N, Bond DA, Farooq U, Saad M, Evens AM, Pandya K, Naik SG, Kamdar M, Haverkos B, Karmali R, Oh TS, Vose JM, Nutsch H, Rubinstein PG, Chaudhry A, Olszewski AJ
Blood Adv 2023 Nov 14;7(21):6381-6394. doi: 10.1182/bloodadvances.2023009731. PMID: 37171397Free PMC Article
Molecular determinants of clinical outcomes in a real-world diffuse large B-cell lymphoma population.
Alduaij W, Collinge B, Ben-Neriah S, Jiang A, Hilton LK, Boyle M, Meissner B, Chong L, Miyata-Takata T, Slack GW, Farinha P, Craig JW, Lytle A, Savage KJ, Villa D, Gerrie AS, Freeman CL, Gascoyne RD, Connors JM, Morin RD, Sehn LH, Mungall AJ, Steidl C, Scott DW
Blood 2023 May 18;141(20):2493-2507. doi: 10.1182/blood.2022018248. PMID: 36302166
Distinct molecular profile of IRF4-rearranged large B-cell lymphoma.
Ramis-Zaldivar JE, Gonzalez-Farré B, Balagué O, Celis V, Nadeu F, Salmerón-Villalobos J, Andrés M, Martin-Guerrero I, Garrido-Pontnou M, Gaafar A, Suñol M, Bárcena C, Garcia-Bragado F, Andión M, Azorín D, Astigarraga I, Sagaseta de Ilurdoz M, Sábado C, Gallego S, Verdú-Amorós J, Fernandez-Delgado R, Perez V, Tapia G, Mozos A, Torrent M, Solano-Páez P, Rivas-Delgado A, Dlouhy I, Clot G, Enjuanes A, López-Guillermo A, Galera P, Oberley MJ, Maguire A, Ramsower C, Rimsza LM, Quintanilla-Martinez L, Jaffe ES, Campo E, Salaverria I
Blood 2020 Jan 23;135(4):274-286. doi: 10.1182/blood.2019002699. PMID: 31738823Free PMC Article

Diagnosis

High-grade B-cell lymphoma, not otherwise specified: CNS involvement and outcomes in a multi-institutional series.
Epperla N, Zayac AS, Landsburg DJ, Bock AM, Nowakowski GS, Ayers EC, Girton M, Hu M, Beckman A, Li S, Medeiros LJ, Chang JE, Kurt H, Sandoval-Sus J, Ansari-Lari MA, Kothari SK, Kress A, Xu ML, Torka P, Sundaram S, Smith SD, Naresh KN, Karimi Y, Bond DA, Evens AM, Naik SG, Kamdar M, Haverkos BM, Karmali R, Farooq U, Vose JM, Rubinstein P, Chaudhry A, Olszewski AJ
Blood Adv 2024 Oct 22;8(20):5355-5364. doi: 10.1182/bloodadvances.2024013791. PMID: 39189932
High-grade B-cell lymphoma, not otherwise specified: a multi-institutional retrospective study.
Zayac AS, Landsburg DJ, Hughes ME, Bock AM, Nowakowski GS, Ayers EC, Girton M, Hu M, Beckman AK, Li S, Medeiros LJ, Chang JE, Stepanovic A, Kurt H, Sandoval-Sus J, Ansari-Lari MA, Kothari SK, Kress A, Xu ML, Torka P, Sundaram S, Smith SD, Naresh KN, Karimi YH, Epperla N, Bond DA, Farooq U, Saad M, Evens AM, Pandya K, Naik SG, Kamdar M, Haverkos B, Karmali R, Oh TS, Vose JM, Nutsch H, Rubinstein PG, Chaudhry A, Olszewski AJ
Blood Adv 2023 Nov 14;7(21):6381-6394. doi: 10.1182/bloodadvances.2023009731. PMID: 37171397Free PMC Article
Molecular determinants of clinical outcomes in a real-world diffuse large B-cell lymphoma population.
Alduaij W, Collinge B, Ben-Neriah S, Jiang A, Hilton LK, Boyle M, Meissner B, Chong L, Miyata-Takata T, Slack GW, Farinha P, Craig JW, Lytle A, Savage KJ, Villa D, Gerrie AS, Freeman CL, Gascoyne RD, Connors JM, Morin RD, Sehn LH, Mungall AJ, Steidl C, Scott DW
Blood 2023 May 18;141(20):2493-2507. doi: 10.1182/blood.2022018248. PMID: 36302166
Defining and treating high-grade B-cell lymphoma, NOS.
Olszewski AJ, Kurt H, Evens AM
Blood 2022 Sep 1;140(9):943-954. doi: 10.1182/blood.2020008374. PMID: 34525177
Distinct molecular profile of IRF4-rearranged large B-cell lymphoma.
Ramis-Zaldivar JE, Gonzalez-Farré B, Balagué O, Celis V, Nadeu F, Salmerón-Villalobos J, Andrés M, Martin-Guerrero I, Garrido-Pontnou M, Gaafar A, Suñol M, Bárcena C, Garcia-Bragado F, Andión M, Azorín D, Astigarraga I, Sagaseta de Ilurdoz M, Sábado C, Gallego S, Verdú-Amorós J, Fernandez-Delgado R, Perez V, Tapia G, Mozos A, Torrent M, Solano-Páez P, Rivas-Delgado A, Dlouhy I, Clot G, Enjuanes A, López-Guillermo A, Galera P, Oberley MJ, Maguire A, Ramsower C, Rimsza LM, Quintanilla-Martinez L, Jaffe ES, Campo E, Salaverria I
Blood 2020 Jan 23;135(4):274-286. doi: 10.1182/blood.2019002699. PMID: 31738823Free PMC Article

Therapy

Epcoritamab: First Approval.
Frampton JE
Drugs 2023 Sep;83(14):1331-1340. doi: 10.1007/s40265-023-01930-4. PMID: 37597091
High-grade B-cell lymphoma, not otherwise specified: a multi-institutional retrospective study.
Zayac AS, Landsburg DJ, Hughes ME, Bock AM, Nowakowski GS, Ayers EC, Girton M, Hu M, Beckman AK, Li S, Medeiros LJ, Chang JE, Stepanovic A, Kurt H, Sandoval-Sus J, Ansari-Lari MA, Kothari SK, Kress A, Xu ML, Torka P, Sundaram S, Smith SD, Naresh KN, Karimi YH, Epperla N, Bond DA, Farooq U, Saad M, Evens AM, Pandya K, Naik SG, Kamdar M, Haverkos B, Karmali R, Oh TS, Vose JM, Nutsch H, Rubinstein PG, Chaudhry A, Olszewski AJ
Blood Adv 2023 Nov 14;7(21):6381-6394. doi: 10.1182/bloodadvances.2023009731. PMID: 37171397Free PMC Article
Current Treatment of Burkitt Lymphoma and High-Grade B-Cell Lymphomas.
Johnson PC, Abramson JS
Oncology (Williston Park) 2022 Aug 10;36(8):499-505. doi: 10.46883/2022.25920970. PMID: 36001789
Double-hit lymphoma: optimizing therapy.
Dunleavy K
Hematology Am Soc Hematol Educ Program 2021 Dec 10;2021(1):157-163. doi: 10.1182/hematology.2021000247. PMID: 34889402Free PMC Article
Loncastuximab Tesirine: First Approval.
Lee A
Drugs 2021 Jul;81(10):1229-1233. doi: 10.1007/s40265-021-01550-w. PMID: 34143407

Prognosis

High-grade B-cell lymphoma, not otherwise specified: CNS involvement and outcomes in a multi-institutional series.
Epperla N, Zayac AS, Landsburg DJ, Bock AM, Nowakowski GS, Ayers EC, Girton M, Hu M, Beckman A, Li S, Medeiros LJ, Chang JE, Kurt H, Sandoval-Sus J, Ansari-Lari MA, Kothari SK, Kress A, Xu ML, Torka P, Sundaram S, Smith SD, Naresh KN, Karimi Y, Bond DA, Evens AM, Naik SG, Kamdar M, Haverkos BM, Karmali R, Farooq U, Vose JM, Rubinstein P, Chaudhry A, Olszewski AJ
Blood Adv 2024 Oct 22;8(20):5355-5364. doi: 10.1182/bloodadvances.2024013791. PMID: 39189932
High-grade B-cell lymphoma, not otherwise specified: a multi-institutional retrospective study.
Zayac AS, Landsburg DJ, Hughes ME, Bock AM, Nowakowski GS, Ayers EC, Girton M, Hu M, Beckman AK, Li S, Medeiros LJ, Chang JE, Stepanovic A, Kurt H, Sandoval-Sus J, Ansari-Lari MA, Kothari SK, Kress A, Xu ML, Torka P, Sundaram S, Smith SD, Naresh KN, Karimi YH, Epperla N, Bond DA, Farooq U, Saad M, Evens AM, Pandya K, Naik SG, Kamdar M, Haverkos B, Karmali R, Oh TS, Vose JM, Nutsch H, Rubinstein PG, Chaudhry A, Olszewski AJ
Blood Adv 2023 Nov 14;7(21):6381-6394. doi: 10.1182/bloodadvances.2023009731. PMID: 37171397Free PMC Article
Molecular determinants of clinical outcomes in a real-world diffuse large B-cell lymphoma population.
Alduaij W, Collinge B, Ben-Neriah S, Jiang A, Hilton LK, Boyle M, Meissner B, Chong L, Miyata-Takata T, Slack GW, Farinha P, Craig JW, Lytle A, Savage KJ, Villa D, Gerrie AS, Freeman CL, Gascoyne RD, Connors JM, Morin RD, Sehn LH, Mungall AJ, Steidl C, Scott DW
Blood 2023 May 18;141(20):2493-2507. doi: 10.1182/blood.2022018248. PMID: 36302166
Defining and treating high-grade B-cell lymphoma, NOS.
Olszewski AJ, Kurt H, Evens AM
Blood 2022 Sep 1;140(9):943-954. doi: 10.1182/blood.2020008374. PMID: 34525177
Distinct molecular profile of IRF4-rearranged large B-cell lymphoma.
Ramis-Zaldivar JE, Gonzalez-Farré B, Balagué O, Celis V, Nadeu F, Salmerón-Villalobos J, Andrés M, Martin-Guerrero I, Garrido-Pontnou M, Gaafar A, Suñol M, Bárcena C, Garcia-Bragado F, Andión M, Azorín D, Astigarraga I, Sagaseta de Ilurdoz M, Sábado C, Gallego S, Verdú-Amorós J, Fernandez-Delgado R, Perez V, Tapia G, Mozos A, Torrent M, Solano-Páez P, Rivas-Delgado A, Dlouhy I, Clot G, Enjuanes A, López-Guillermo A, Galera P, Oberley MJ, Maguire A, Ramsower C, Rimsza LM, Quintanilla-Martinez L, Jaffe ES, Campo E, Salaverria I
Blood 2020 Jan 23;135(4):274-286. doi: 10.1182/blood.2019002699. PMID: 31738823Free PMC Article

Clinical prediction guides

High-grade B-cell lymphoma, not otherwise specified, presenting as primary peritoneal lymphomatosis and successfully treated with dose-adjusted EPOCH-R.
Fujimi A, Nagamachi Y, Yamauchi N, Onoyama N, Hayasaka N, Matsuno T, Koike K, Goto Y, Ihara K, Kato J, Nishisato T, Kawase H, Yano T, Kanaseki T, Sugita S, Kobune M
J Clin Exp Hematop 2024 Mar 28;64(1):37-44. Epub 2024 Jan 28 doi: 10.3960/jslrt.23044. PMID: 38281744Free PMC Article
High-grade B-cell lymphoma, not otherwise specified: a multi-institutional retrospective study.
Zayac AS, Landsburg DJ, Hughes ME, Bock AM, Nowakowski GS, Ayers EC, Girton M, Hu M, Beckman AK, Li S, Medeiros LJ, Chang JE, Stepanovic A, Kurt H, Sandoval-Sus J, Ansari-Lari MA, Kothari SK, Kress A, Xu ML, Torka P, Sundaram S, Smith SD, Naresh KN, Karimi YH, Epperla N, Bond DA, Farooq U, Saad M, Evens AM, Pandya K, Naik SG, Kamdar M, Haverkos B, Karmali R, Oh TS, Vose JM, Nutsch H, Rubinstein PG, Chaudhry A, Olszewski AJ
Blood Adv 2023 Nov 14;7(21):6381-6394. doi: 10.1182/bloodadvances.2023009731. PMID: 37171397Free PMC Article
Blastoid high-grade B-cell lymphoma initially presenting in bone marrow: a diagnostic challenge.
Khanlari M, Medeiros LJ, Lin P, Xu J, You MJ, Tang G, Yin CC, Wang W, Qiu L, Miranda RN, Bueso-Ramos CE, Li S
Mod Pathol 2022 Mar;35(3):419-426. Epub 2021 Oct 4 doi: 10.1038/s41379-021-00909-4. PMID: 34608246
Non-diffuse large B-cell lymphoma transformation from follicular lymphoma: a single-institution study of 19 cases.
Maeshima AM, Taniguchi H, Ida H, Hosoba R, Fujino T, Saito Y, Yuda S, Makita S, Fukuhara S, Munakata W, Suzuki T, Maruyama D, Izutsu K
Hum Pathol 2020 Aug;102:33-43. Epub 2020 Jun 12 doi: 10.1016/j.humpath.2020.06.001. PMID: 32540222
Colorectal diffuse large B-cell lymphoma: molecular subclassification and prognostic significance of immunoglobulin gene translocation.
Hori Y, Yamamoto H, Nozaki Y, Torisu T, Fujiwara M, Taguchi K, Nishiyama K, Nakamura S, Kitazono T, Oda Y
Hum Pathol 2020 Feb;96:67-78. Epub 2019 Nov 15 doi: 10.1016/j.humpath.2019.09.003. PMID: 31734190

Recent systematic reviews

Cardiovascular adverse events in patients with non-Hodgkin lymphoma treated with first-line cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) or CHOP with rituximab (R-CHOP): a systematic review and meta-analysis.
Linschoten M, Kamphuis JAM, van Rhenen A, Bosman LP, Cramer MJ, Doevendans PA, Teske AJ, Asselbergs FW
Lancet Haematol 2020 Apr;7(4):e295-e308. Epub 2020 Mar 2 doi: 10.1016/S2352-3026(20)30031-4. PMID: 32135128

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