t(1;6)(p35;p25) ?/IRF4
2005-01-01 Jean-Loup Huret   Affiliation1.Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France
Clinics and Pathology
Disease
Chronic lymphocytic leukemia (CLL)
Phenotype stem cell origin
Unmutated status of IgVH (an unfavourable prognostic feature) was found in all cases.
Epidemiology
8 patients to date, representing 0.5% of CLL cases in a country based survey; sex ratio: 6 male/2 female patients, aged 33-81 years (med: 62.5 years).
Clinics
Two cases in Binet stage A, 4 in stage B, 2 in stage C. Normal LDH values in 7 of 8.
Prognosis
Yet unknown (median follow up is only 28 months), but 3 patients developed a diffuse large B-cell lymphoma (DLBCL), and 3 patients (2 of which with DLBCL) died at 29, 76, and 95 months.
Genes Involved and Proteins
Note
IRF4 (6p25.2) is possibly involved in the translocation. The gene in 1p35.3 is unknown.
Highly cited references
| Pubmed ID | Year | Title | Citations |
|---|---|---|---|
| 20192994 | 2010 | IRF4 and its regulators: evolving insights into the pathogenesis of inflammatory arthritis? | 173 |
| 36814912 | 2023 | Regulatory effects of IRF4 on immune cells in the tumor microenvironment. | 132 |
| 37935654 | 2023 | Transcriptional reprogramming by mutated IRF4 in lymphoma. | 116 |
| 39473489 | 2017 | Role of IRF4-Mediated Inflammation: Implication in Neurodegenerative Diseases. | 114 |
| 37755331 | 2023 | Roles of interferon regulatory factor 4 in the AKI-CKD transition, glomerular diseases and kidney allograft rejection. | 107 |
| 36077849 | 2022 | IRF4 as an Oncogenic Master Transcription Factor. | 106 |
| 35472072 | 2022 | Reduced IRF4 expression promotes lytic phenotype in Type 2 EBV-infected B cells. | 96 |
| 39356034 | 2024 | Targeting Immunoproteasome in Polarized Macrophages Ameliorates Experimental Emphysema Via Activating NRF1/2-P62 Axis and Suppressing IRF4 Transcription. | 95 |
| 37697097 | 2023 | Control of nutrient uptake by IRF4 orchestrates innate immune memory. | 93 |
| 38880659 | 2024 | Interferon regulatory factor 4 modulates epigenetic silencing and cancer-critical pathways in melanoma cells. | 93 |
| 35504924 | 2022 | IRF4 drives clonal evolution and lineage choice in a zebrafish model of T-cell lymphoma. | 92 |
| 19383829 | 2009 | IRF4: Immunity. Malignancy! Therapy? | 87 |
| 33406405 | 2021 | Acetyl-CoA Synthetase 2: A Critical Linkage in Obesity-Induced Tumorigenesis in Myeloma. | 85 |
| 37767768 | 2024 | Transcriptional Heterogeneity Overcomes Super-Enhancer Disrupting Drug Combinations in Multiple Myeloma. | 84 |
| 29537367 | 2018 | IRF4 haploinsufficiency in a family with Whipple's disease. | 82 |
| 34282330 | 2021 | BATF and IRF4 cooperate to counter exhaustion in tumor-infiltrating CAR T cells. | 80 |
| 34310776 | 2021 | Impact of hypoxia on the pathogenesis and therapy resistance in multiple myeloma. | 80 |
| 38906156 | 2024 | IRF4 requires ARID1A to establish plasma cell identity in multiple myeloma. | 78 |
| 31949143 | 2020 | Genomic programming of IRF4-expressing human Langerhans cells. | 78 |
| 37432594 | 2024 | Central IRF4/5 Signaling Are Critical for Microglial Activation and Impact on Stroke Outcomes. | 78 |
| 36662884 | 2023 | A multimorphic mutation in IRF4 causes human autosomal dominant combined immunodeficiency. | 77 |
| 33476575 | 2021 | Selective antisense oligonucleotide inhibition of human IRF4 prevents malignant myeloma regeneration via cell cycle disruption. | 77 |
| 37809068 | 2023 | Human inborn errors of immunity associated with IRF4. | 75 |
| 36495369 | 2023 | The dynamic functions of IRF4 in B cell malignancies. | 75 |
| 38457926 | 2024 | C-terminal binding protein 2 is a novel tumor suppressor targeting the MYC-IRF4 axis in multiple myeloma. | 75 |
| 36182745 | 2022 | Druggable host gene dependencies in primary effusion lymphoma. | 74 |
| 32125291 | 2020 | IRF4 instructs effector Treg differentiation and immune suppression in human cancer. | 73 |
| 38605225 | 2024 | Accelerated plasma-cell differentiation in Bach2-deficient mouse B cells is caused by altered IRF4 functions. | 72 |
| 32795402 | 2020 | High Amount of Transcription Factor IRF8 Engages AP1-IRF Composite Elements in Enhancers to Direct Type 1 Conventional Dendritic Cell Identity. | 72 |
| 33042761 | 2020 | IRF4 in Skeletal Muscle Regulates Exercise Capacity via PTG/Glycogen Pathway. | 67 |
| 35963621 | 2022 | Regulation of microglial activation in stroke in aged mice: a translational study. | 66 |
| 26728187 | 2016 | The KDM3A-KLF2-IRF4 axis maintains myeloma cell survival. | 65 |
| 35059038 | 2021 | Molecular interactions of IRF4 in B cell development and malignancies. | 63 |
| 39003776 | 2024 | A dual-gene panel of two fragments of methylated IRF4 and one of ZEB2 in plasma cell-free DNA for gastric cancer detection. | 62 |
| 37770480 | 2023 | Metabolic crosstalk between skeletal muscle cells and liver through IRF4-FSTL1 in nonalcoholic steatohepatitis. | 60 |
| 30078553 | 2018 | Brown Adipose Tissue Controls Skeletal Muscle Function via the Secretion of Myostatin. | 60 |
| 38491484 | 2024 | IRF4-mediated Treg phenotype switching can aggravate hyperoxia-induced alveolar epithelial cell injury. | 58 |
| 34759017 | 2021 | B Cell-Intrinsic IRF4 Haploinsufficiency Impairs Affinity Maturation. | 58 |
| 36194494 | 2022 | IRF4 expression by lung dendritic cells drives acute but not Trm cell-dependent memory Th2 responses. | 58 |
| 24995979 | 2014 | IRF4 is a key thermogenic transcriptional partner of PGC-1α. | 57 |
| 37469513 | 2023 | IRF4 is required for migration of CD4(+) T cells to the intestine but not for Th2 and Th17 cell maintenance. | 54 |
| 36917008 | 2023 | A neomorphic mutation in the interferon activation domain of IRF4 causes a dominant primary immunodeficiency. | 51 |
| 18775669 | 2008 | Interferon regulatory factor 4 and 8 in B-cell development. | 51 |
| 31738823 | 2020 | Distinct molecular profile of IRF4-rearranged large B-cell lymphoma. | 50 |
| 29221730 | 2017 | Ablation of Transcription Factor IRF4 Promotes Transplant Acceptance by Driving Allogenic CD4(+) T Cell Dysfunction. | 50 |
| 36159833 | 2022 | Jmjd3/IRF4 axis aggravates myeloid fibroblast activation and m2 macrophage to myofibroblast transition in renal fibrosis. | 50 |
| 30586599 | 2019 | Myeloid cell IRF4 signaling protects neonatal brains from hypoxic ischemic encephalopathy. | 49 |
| 39501302 | 2024 | METTL3 facilitates kidney injury through promoting IRF4-mediated plasma cell infiltration via an m6A-dependent manner in systemic lupus erythematosus. | 47 |
| 23926303 | 2013 | A role for IRF4 in the development of CLL. | 47 |
| 36129197 | 2023 | Novel antimyeloma therapeutic option with inhibition of the HDAC1-IRF4 axis and PIM kinase. | 47 |
| 33533913 | 2021 | Structural determinants of the IRF4/DNA homodimeric complex. | 46 |
| 37287982 | 2023 | IRF4 downregulation improves sensitivity and endurance of CAR T cell functional capacities. | 46 |
| 38195465 | 2024 | IRF4 affects the protective effect of regulatory T cells on the pulmonary vasculature of a bronchopulmonary dysplasia mouse model by regulating FOXP3. | 45 |
| 34728538 | 2021 | SOX10 Regulates Melanoma Immunogenicity through an IRF4-IRF1 Axis. | 43 |
| 38178902 | 2023 | Continuous Expression of Interferon Regulatory Factor 4 Sustains CD8(+) T Cell Immunity against Tumor. | 40 |
| 37860697 | 2023 | The transcription factor IRF4 determines the anti-tumor immunity of CD8(+) T cells. | 39 |
| 35338347 | 2023 | SUMOylation inhibition enhances multiple myeloma sensitivity to lenalidomide. | 39 |
| 24510125 | 2014 | IRF4 is a novel mediator for neuronal survival in ischaemic stroke. | 39 |
| 22003200 | 2011 | MicroRNA-125b potentiates macrophage activation. | 38 |
| 35713959 | 2022 | Obese Skeletal Muscle-Expressed Interferon Regulatory Factor 4 Transcriptionally Regulates Mitochondrial Branched-Chain Aminotransferase Reprogramming Metabolome. | 35 |
| 34913532 | 2022 | The molecular basis for the development of adult T-cell leukemia/lymphoma in patients with an IRF4(K59R) mutation. | 31 |
| 37081786 | 2023 | IRF4 rearrangement may predict favorable prognosis in children and young adults with primary head and neck large B-cell lymphoma. | 30 |
| 34253454 | 2021 | IRF4 ablation in B cells abrogates allogeneic B cell responses and prevents chronic transplant rejection. | 30 |
| 18568025 | 2008 | IRF4 addiction in multiple myeloma. | 29 |
| 35397366 | 2022 | IRF4 suppresses osteogenic differentiation of BM-MSCs by transcriptionally activating miR-636/DOCK9 axis. | 29 |
| 39123188 | 2024 | Suppression of SENP3 enhances macrophage alternative activation by mediating IRF4 de-SUMOylation in ESCC progression. | 28 |
| 34823176 | 2022 | Identification and antiviral effect of Cherry Valley duck IRF4. | 28 |
| 35993451 | 2022 | Postbiotics engage IRF4 in adipocytes to promote sex-dependent changes in blood glucose during obesity. | 24 |
| 36810796 | 2023 | Prevalence of IRF4 rearrangement in large B-cell lymphomas of the Waldeyer's ring in adults. | 24 |
| 21791429 | 2011 | IRF4 mutations in chronic lymphocytic leukemia. | 15 |
| 37211558 | 2023 | Repression of interferon regulatory factor-4 (IRF4) hyperactivation restricts murine lupus. | 4 |
| 29246443 | 2017 | Transcription Factor IRF4 Promotes CD8(+) T Cell Exhaustion and Limits the Development of Memory-like T Cells during Chronic Infection. | 0 |
| 20182347 | 2010 | MUM1/IRF4: A Review. | 0 |
| 35908629 | 2022 | Lineage- and Stage-Specific Oncogenicity of IRF4. | 0 |
| 38615379 | 2024 | Roles of IRF4 in various immune cells in systemic lupus erythematosus. | 0 |
| 38969872 | 2024 | Epigenetic recording of stimulation history reveals BLIMP1-BACH2 balance in determining memory B cell fate upon recall challenge. | 0 |
| 30098518 | 2018 | IRF4 in multiple myeloma-Biology, disease and therapeutic target. | 0 |
| 38943641 | 2024 | IRF4 impedes human CD8 T cell function and promotes cell proliferation and PD-1 expression. | 0 |
| 23010632 | 2012 | Targeting IRF4 in autoimmune diseases. | 0 |
| 22500833 | 2012 | The diverse roles of IRF4 in late germinal center B-cell differentiation. | 0 |
| 36081178 | 2022 | Regulatory network of BLIMP1, IRF4, and XBP1 triad in plasmacytic differentiation and multiple myeloma pathogenesis. | 0 |
| 24782159 | 2014 | IRF4 at the crossroads of effector T-cell fate decision. | 0 |
| 38164749 | 2024 | IRF4 induces M1 macrophage polarization and aggravates ulcerative colitis progression by the Bcl6-dependent STAT3 pathway. | 0 |
| 27826752 | 2016 | Essential role of interferon regulatory factor 4 (IRF4) in immune cell development. | 0 |
| 39172106 | 2024 | Clinicopathological and Molecular Characteristics of Rare EBV-associated Diffuse Large B-cell Lymphoma With IRF4 Rearrangement. | 0 |
| 36944579 | 2023 | Lymphomatoid papulosis with DUSP22-IRF4 rearrangement: A case report and literature review. | 0 |
| 37385124 | 2023 | Inhibition of IL-4/STAT6/IRF4 signaling reduces the epithelial-mesenchymal transition in eosinophilic chronic rhinosinusitis with nasal polyps. | 0 |
| 39437487 | 2024 | IRF4: A potential prognostic biomarker for immunotherapy in NSCLC. | 0 |
| 36265015 | 2022 | The Double Face of IRF4 in Metabolic Reprogramming. | 0 |
| 37634651 | 2023 | Follicular lymphoma and diffuse large B-cell lymphoma with BCL2 and IRF4 rearrangements in adult patients. | 0 |
| 28103633 | 2017 | IRF4 rs12203592 functional variant and melanoma survival. | 0 |
| 34117720 | 2022 | The Immunity-malignancy equilibrium in multiple myeloma: lessons from oncogenic events in plasma cells. | 0 |
| 30218581 | 2019 | A hyperactive mutant of interferon-regulatory factor 4. | 0 |
| 33623139 | 2021 | IRF4 modulates the response to BCR activation in chronic lymphocytic leukemia regulating IKAROS and SYK. | 0 |
| 11846983 | 2002 | The role of IRF-4 in transcriptional regulation. | 0 |
| 31972002 | 2020 | Feed-forward regulatory loop driven by IRF4 and NF-κB in adult T-cell leukemia/lymphoma. | 0 |
| 37962684 | 2024 | Large B-cell lymphoma with IRF4 rearrangement: a multi-centric study with focus on potential misleading phenotypes. | 0 |
| 30907723 | 2019 | IRF4 promotes Epstein-Barr virus activation in Burkitt's lymphoma cells. | 0 |
| 27601624 | 2016 | Interferon regulatory factor 4 (IRF4) controls myeloid-derived suppressor cell (MDSC) differentiation and function. | 0 |
| 34670117 | 2022 | RNF2 promotes the progression of colon cancer by regulating ubiquitination and degradation of IRF4. | 0 |
Article Bibliography
| Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|
| 15510210 | 2005 | Translocation t(1;6)(p35.3;p25.2): a new recurrent aberration in "unmutated" B-CLL. | Michaux L et al |
Summary

t(1;6)(p35;p25) G- banding - top: Courtesy Melanie Zenger and Claudia Haferlach; bottom Courtesy Aurelia Meloni-Ehrig.
Citation
Jean-Loup Huret
t(1;6)(p35;p25) ?/IRF4
Atlas Genet Cytogenet Oncol Haematol. 2005-01-01
Online version: http://atlasgeneticsoncology.org/haematological/1378/t(1;6)(p35;p25)-irf4
