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B-cell lymphoma, unclassifiable, with features intermediate between diffuse large b-cell lymphoma and classical Hodgkin lymphoma

MedGen UID:
234323
•Concept ID:
C1333878
•
Neoplastic Process
Synonyms: B-cell lymphoma unclassifiable with features intermediate between classical Hodgkin lymphoma and diffuse large B-cell lymphoma; B-Cell Lymphoma, Unclassifiable, with Features Intermediate between Diffuse Large B-Cell Lymphoma and Classic Hodgkin Lymphoma; B-cell lymphoma, unclassifiable, with features Intermediate between diffuse large B-cell lymphoma and Classic Hodgkin lymphoma; B-Cell Lymphoma, Unclassifiable, with Features Intermediate between Diffuse Large B-Cell Lymphoma and Classical Hodgkin Lymphoma; B-cell lymphoma, unclassifiable, with features Intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma; Gray Zone Lymphoma; Gray zone lymphoma; Grey zone lymphoma; Hodgkin-Like Anaplastic Large Cell Lymphoma; Hodgkin-like anaplastic large cell lymphoma; Large B-Cell Lymphoma with Hodgkin Features; large B-cell lymphoma with Hodgkin features
SNOMED CT: B-cell lymphoma unclassifiable with features intermediate between classical Hodgkin lymphoma and diffuse large B-cell lymphoma (722954005)
 
Monarch Initiative: MONDO:0003658

Definition

A group of lymphomas displaying molecular, morphologic, immunophenotypic, and clinical overlap between classic Hodgkin lymphoma and diffuse large B-cell lymphoma. This term particularly applies to mediastinal lymphomas with overlapping features of mediastinal (thymic) large B-cell lymphoma and classic Hodgkin lymphoma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVB-cell lymphoma, unclassifiable, with features intermediate between diffuse large b-cell lymphoma and classical Hodgkin lymphoma

Professional guidelines

PubMed

Utility of Combined EZH2, p-ERK1/2, p-STAT, and MYC Expression in the Differential Diagnosis of EZH2-positive Hodgkin Lymphomas and Related Large B-Cell Lymphomas.
Tian X, Xu J, Dorfman DM
Am J Surg Pathol 2019 Jan;43(1):102-109. doi: 10.1097/PAS.0000000000001180. PMID: 30371509

Recent clinical studies

Etiology

Comparative pathologic analysis of mediastinal B-cell lymphomas: selective expression of p63 but no GATA3 optimally differentiates primary mediastinal large B-cell lymphoma from classic Hodgkin lymphoma.
Kim HJ, Kim HK, Park G, Min SK, Cha HJ, Lee H, Choi SJ, Na HY, Choe JY, Kim JE
Diagn Pathol 2019 Dec 12;14(1):133. doi: 10.1186/s13000-019-0918-x. PMID: 31831043Free PMC Article
Early post-transplant lymphoproliferative disorder – Case of fatal lymphoma after kidney transplantation.
Laušević M, Markovic-Lipkovski J, Terzić T, Jovanović N, Milinković M, Naumović R
Srp Arh Celok Lek 2016 May-Jun;144(5-6):325-8. PMID: 29648755
Distinguishing Classical Hodgkin Lymphoma, Gray Zone Lymphoma, and Large B-cell Lymphoma: A Proposed Scoring System.
O'Malley DP, Fedoriw Y, Weiss LM
Appl Immunohistochem Mol Morphol 2016 Sep;24(8):535-40. doi: 10.1097/PAI.0000000000000236. PMID: 26447896
Rituximab in combination with CODOX-M/IVAC: a retrospective analysis of 23 cases of non-HIV related B-cell non-Hodgkin lymphoma with proliferation index >95%.
Mohamedbhai SG, Sibson K, Marafioti T, Kayani I, Lowry L, Goldstone AH, Linch DC, Ardeshna KM
Br J Haematol 2011 Jan;152(2):175-81. Epub 2010 Nov 24 doi: 10.1111/j.1365-2141.2010.08447.x. PMID: 21092025
B-cell lymphomas with features intermediate between distinct pathologic entities. From pathogenesis to pathology.
Carbone A, Gloghini A, Aiello A, Testi A, Cabras A
Hum Pathol 2010 May;41(5):621-31. doi: 10.1016/j.humpath.2009.10.027. PMID: 20398809

Diagnosis

Mediastinal large B cell lymphoma and surrounding gray areas: a report of the lymphoma workshop of the 20th meeting of the European Association for Haematopathology.
Gibson SE, Dojcinov S, Dotlic S, Hartmann S, Hsi ED, Klimkowska M, Melle F, Pileri SA, Ramsower CA, Rech K, Rimsza LM, Rodriguez-Pinilla SM, Tousseyn TA, de Jong D, Sabattini E
Virchows Arch 2023 Dec;483(6):733-749. Epub 2023 Aug 2 doi: 10.1007/s00428-023-03550-5. PMID: 37530791Free PMC Article
Evolution in the definition and diagnosis of the Hodgkin lymphomas and related entities.
Tousseyn TA, King RL, Fend F, Feldman AL, Brousset P, Jaffe ES
Virchows Arch 2023 Jan;482(1):207-226. Epub 2022 Oct 24 doi: 10.1007/s00428-022-03427-z. PMID: 36274093
Challenges in the Diagnosis of Gray Zone Lymphomas.
Parker K, Venkataraman G
Surg Pathol Clin 2019 Sep;12(3):709-718. Epub 2019 May 23 doi: 10.1016/j.path.2019.03.014. PMID: 31352983
Distinguishing Classical Hodgkin Lymphoma, Gray Zone Lymphoma, and Large B-cell Lymphoma: A Proposed Scoring System.
O'Malley DP, Fedoriw Y, Weiss LM
Appl Immunohistochem Mol Morphol 2016 Sep;24(8):535-40. doi: 10.1097/PAI.0000000000000236. PMID: 26447896
B-cell lymphomas with features intermediate between distinct pathologic entities. From pathogenesis to pathology.
Carbone A, Gloghini A, Aiello A, Testi A, Cabras A
Hum Pathol 2010 May;41(5):621-31. doi: 10.1016/j.humpath.2009.10.027. PMID: 20398809

Therapy

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
"Do Not Ignore Musculoskeletal Pain"-Initial Presentation of a Rare Malignant Disease: Case Report and Literature Review.
Patel I, Akoluk A, Upadhyaya V, Makadia S, Douedi S, Farooq T, Flynn D, Levitt M, Hossain MA
J Investig Med High Impact Case Rep 2020 Jan-Dec;8:2324709620941316. doi: 10.1177/2324709620941316. PMID: 32660277Free PMC Article
How I manage patients with grey zone lymphoma.
Kritharis A, Pilichowska M, Evens AM
Br J Haematol 2016 Aug;174(3):345-50. Epub 2016 Jun 15 doi: 10.1111/bjh.14174. PMID: 27301470
Gray zone lymphoma: better treated like hodgkin lymphoma or mediastinal large B-cell lymphoma?
Dunleavy K, Grant C, Eberle FC, Pittaluga S, Jaffe ES, Wilson WH
Curr Hematol Malig Rep 2012 Sep;7(3):241-7. doi: 10.1007/s11899-012-0130-5. PMID: 22833351Free PMC Article
The spectrum of B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma: a description of 10 cases.
Gualco G, Natkunam Y, Bacchi CE
Mod Pathol 2012 May;25(5):661-74. Epub 2012 Jan 6 doi: 10.1038/modpathol.2011.200. PMID: 22222636

Prognosis

Diagnostic utility of STAT6(YE361) expression in classical Hodgkin lymphoma and related entities.
Van Slambrouck C, Huh J, Suh C, Song JY, Menon MP, Sohani AR, Duffield AS, Goldberg RC, Dama P, Kiyotani K, Godfrey J, Fitzpatrick C, Kline J, Smith SM, Jaffe ES, Hartmann S, Venkataraman G
Mod Pathol 2020 May;33(5):834-845. Epub 2019 Dec 10 doi: 10.1038/s41379-019-0428-0. PMID: 31822802Free PMC Article
Challenges in the Diagnosis of Gray Zone Lymphomas.
Parker K, Venkataraman G
Surg Pathol Clin 2019 Sep;12(3):709-718. Epub 2019 May 23 doi: 10.1016/j.path.2019.03.014. PMID: 31352983
Gray-zone lymphoma. Examples of rare clinical manifestation.
Misyurina AE, Kravchenko SK, Mangasarova YK, Magomedova AU, Kovrigina AM, Shimanovskaya LT, Fastova EA, Misyurin VA, Model SV, Chebotarev DI, Kostina IE, Savchenko VG
Ter Arkh 2019 May 16;91(4):107-113. doi: 10.26442/00403660.2019.04.000071. PMID: 31094484
J chain and myocyte enhancer factor 2B are useful in differentiating classical Hodgkin lymphoma from nodular lymphocyte predominant Hodgkin lymphoma and primary mediastinal large B-cell lymphoma.
Moore EM, Swerdlow SH, Gibson SE
Hum Pathol 2017 Oct;68:47-53. Epub 2017 Aug 26 doi: 10.1016/j.humpath.2017.08.015. PMID: 28851661
How I manage patients with grey zone lymphoma.
Kritharis A, Pilichowska M, Evens AM
Br J Haematol 2016 Aug;174(3):345-50. Epub 2016 Jun 15 doi: 10.1111/bjh.14174. PMID: 27301470

Clinical prediction guides

Diagnostic utility of STAT6(YE361) expression in classical Hodgkin lymphoma and related entities.
Van Slambrouck C, Huh J, Suh C, Song JY, Menon MP, Sohani AR, Duffield AS, Goldberg RC, Dama P, Kiyotani K, Godfrey J, Fitzpatrick C, Kline J, Smith SM, Jaffe ES, Hartmann S, Venkataraman G
Mod Pathol 2020 May;33(5):834-845. Epub 2019 Dec 10 doi: 10.1038/s41379-019-0428-0. PMID: 31822802Free PMC Article
J chain and myocyte enhancer factor 2B are useful in differentiating classical Hodgkin lymphoma from nodular lymphocyte predominant Hodgkin lymphoma and primary mediastinal large B-cell lymphoma.
Moore EM, Swerdlow SH, Gibson SE
Hum Pathol 2017 Oct;68:47-53. Epub 2017 Aug 26 doi: 10.1016/j.humpath.2017.08.015. PMID: 28851661
Distinguishing Classical Hodgkin Lymphoma, Gray Zone Lymphoma, and Large B-cell Lymphoma: A Proposed Scoring System.
O'Malley DP, Fedoriw Y, Weiss LM
Appl Immunohistochem Mol Morphol 2016 Sep;24(8):535-40. doi: 10.1097/PAI.0000000000000236. PMID: 26447896
BOB.1, CD79a and cyclin E are the most appropriate markers to discriminate classical Hodgkin's lymphoma from primary mediastinal large B-cell lymphoma.
Hoeller S, Zihler D, Zlobec I, Obermann EC, Pileri SA, Dirnhofer S, Tzankov A
Histopathology 2010 Jan;56(2):217-28. doi: 10.1111/j.1365-2559.2009.03462.x. PMID: 20102401

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