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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

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

Term Hierarchy

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

Professional guidelines

PubMed

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
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

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
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
Dunleavy K
Hematology Am Soc Hematol Educ Program 2021 Dec 10;2021(1):157-163. doi: 10.1182/hematology.2021000247. PMID: 34889402Free PMC Article
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
Ok CY, Medeiros LJ
Pathology 2020 Jan;52(1):68-77. Epub 2019 Nov 15 doi: 10.1016/j.pathol.2019.09.008. PMID: 31735344

Diagnosis

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
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
Olszewski AJ, Kurt H, Evens AM
Blood 2022 Sep 1;140(9):943-954. doi: 10.1182/blood.2020008374. PMID: 34525177
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
Ok CY, Medeiros LJ
Pathology 2020 Jan;52(1):68-77. Epub 2019 Nov 15 doi: 10.1016/j.pathol.2019.09.008. PMID: 31735344

Therapy

Frampton JE
Drugs 2023 Sep;83(14):1331-1340. doi: 10.1007/s40265-023-01930-4. PMID: 37597091
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
Johnson PC, Abramson JS
Oncology (Williston Park) 2022 Aug 10;36(8):499-505. doi: 10.46883/2022.25920970. PMID: 36001789
Dunleavy K
Hematology Am Soc Hematol Educ Program 2021 Dec 10;2021(1):157-163. doi: 10.1182/hematology.2021000247. PMID: 34889402Free PMC Article
Lee A
Drugs 2021 Jul;81(10):1229-1233. doi: 10.1007/s40265-021-01550-w. PMID: 34143407

Prognosis

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
Li S, Qiu L, Xu J, Lin P, Ok CY, Tang G, McDonnell TJ, James You M, Khanlari M, Miranda RN, Medeiros LJ
Leukemia 2023 Feb;37(2):422-432. Epub 2022 Dec 13 doi: 10.1038/s41375-022-01778-9. PMID: 36513804
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
Olszewski AJ, Kurt H, Evens AM
Blood 2022 Sep 1;140(9):943-954. doi: 10.1182/blood.2020008374. PMID: 34525177
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

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: 38281744
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
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
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
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

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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