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Large B-Cell Lymphoma with IRF4 Rearrangement

MedGen UID:
1616175
•Concept ID:
C4524676
•
Neoplastic Process
Synonyms: Large B-cell lymphoma with interferon regulatory factor 4 rearrangement; Large B-cell lymphoma with IRF4 rearrangement; LBCL with IRF4 Rearrangement
SNOMED CT: Large B-cell lymphoma with interferon regulatory factor 4 rearrangement (786960000); Large B-cell lymphoma with IRF4 rearrangement (786960000)

Definition

A large B-cell lymphoma typically affecting the Waldeyer ring and/or cervical lymph nodes. It occurs most commonly in children and young adults and is low stage. It is characterized by a follicular, follicular and diffuse, or pure diffuse growth pattern resembling follicular lymphoma grade 3B or a diffuse large B-cell lymphoma. Most cases have IG/IRF4 rearrangements. BCL2 rearrangements are not present. Some cases that belong in this category lack an IRF4 rearrangement but have strong IRF4/MUM1 expression. This lymphoma is more aggressive than pediatric-type follicular lymphomas. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLarge B-Cell Lymphoma with IRF4 Rearrangement

Recent clinical studies

Etiology

Aggressive B-cell non-Hodgkin lymphomas: a report of the lymphoma workshop of the 20th meeting of the European Association for Haematopathology.
Rodriguez-Pinilla SM, Dojcinov S, Dotlic S, Gibson SE, Hartmann S, Klimkowska M, Sabattini E, Tousseyn TA, de Jong D, Hsi ED
Virchows Arch 2024 Jan;484(1):15-29. Epub 2023 Aug 2 doi: 10.1007/s00428-023-03579-6. PMID: 37530792Free PMC Article
New developments in non-Hodgkin lymphoid malignancies.
Ganapathi KA, Brown LE, Prakash S, Bhargava P
Pathology 2021 Apr;53(3):349-366. Epub 2021 Mar 5 doi: 10.1016/j.pathol.2021.01.002. PMID: 33685720
Application of 2016 WHO classification in the diagnosis of paediatric high-grade MYC-negative mature B-cell lymphoma with Burkitt-like morphological features.
Zhang L, Brown LE, Bowen LM, McCarthy LC, Cooley LD, Repnikova E, Gener MA, Garola R, August KJ, Hays JA, Zwick DL, Li W
J Clin Pathol 2020 Sep;73(9):563-570. Epub 2020 Jan 21 doi: 10.1136/jclinpath-2019-206267. PMID: 31964683

Diagnosis

Large B-cell lymphoma with IRF4 rearrangement: a multi-centric study with focus on potential misleading phenotypes.
Pizzi M, Bongiovanni L, Lorenzi L, Righi S, Scarmozzino F, Balzarini P, Santoro L, Mussolin L, Carraro E, Pillon M, Bonaldi L, Vianello F, Agostinelli C, Ponzoni M, Dei Tos AP, Sabattini E
Virchows Arch 2024 Mar;484(3):521-526. Epub 2023 Nov 14 doi: 10.1007/s00428-023-03689-1. PMID: 37962684
Aggressive B-cell non-Hodgkin lymphomas: a report of the lymphoma workshop of the 20th meeting of the European Association for Haematopathology.
Rodriguez-Pinilla SM, Dojcinov S, Dotlic S, Gibson SE, Hartmann S, Klimkowska M, Sabattini E, Tousseyn TA, de Jong D, Hsi ED
Virchows Arch 2024 Jan;484(1):15-29. Epub 2023 Aug 2 doi: 10.1007/s00428-023-03579-6. PMID: 37530792Free PMC Article
New developments in non-Hodgkin lymphoid malignancies.
Ganapathi KA, Brown LE, Prakash S, Bhargava P
Pathology 2021 Apr;53(3):349-366. Epub 2021 Mar 5 doi: 10.1016/j.pathol.2021.01.002. PMID: 33685720
Application of 2016 WHO classification in the diagnosis of paediatric high-grade MYC-negative mature B-cell lymphoma with Burkitt-like morphological features.
Zhang L, Brown LE, Bowen LM, McCarthy LC, Cooley LD, Repnikova E, Gener MA, Garola R, August KJ, Hays JA, Zwick DL, Li W
J Clin Pathol 2020 Sep;73(9):563-570. Epub 2020 Jan 21 doi: 10.1136/jclinpath-2019-206267. PMID: 31964683

Clinical prediction guides

Follicular lymphoma and diffuse large B-cell lymphoma with BCL2 and IRF4 rearrangements in adult patients.
Yuan J, Liu H, Hu S, Miranda RN, Xu X, Bayerl MG, Artymiuk CJ, Berg H, King RL, Shi M, He R, Viswanatha D, Medeiros LJ, McPhail ED
Hum Pathol 2023 Nov;141:22-29. Epub 2023 Aug 25 doi: 10.1016/j.humpath.2023.08.008. PMID: 37634651

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