t(6;14)(q25-27;q32) BCL11B/?

2008-07-01   Jean-Loup Huret 

1.Genetics, Dept Medical Information, University of Poitiers; CHU Poitiers Hospital, F-86021 Poitiers, France

Clinics and Pathology


Haematological malignancies

Phenotype stem cell origin

The phenotype is variable, and the translocation may be heterogenous at the level of the genes involved; there was one acute lymphoblastic leukaemia (ALL), four biphenotypic acute leukaemias (BAL), two of which with expression of myeloid and T-cell markers, one acute myeloid leukaemia (AML), one chronic T-cell neoplasm, and one chronic lymphocytic leukaemia (B-CLL) Binet stage B.


Only 8 cases to date (5M/2F); there was 3 children and 4 adults (ages were: 12, 14, 28, 41, 54, 62, ?, ?).


Survival data is available in only 5 cases; patients died: "shortly", at 17 months, 33 mths, 45 mths, and 112 mths after diagnosis.

Genes Involved and Proteins

In only one of the above mentioned cases, BCL11B was detected as being involved in the translocation; the partner is unknown (Bezrookove et al., 2004). The case with BCL11B involvement was a case of M1-AML in a 54 year old male patient who died shortly after diagnosis. The t(6;14) was the sole anomaly. In other cases, data is missing concerning the genes implicated in the translocation.
Gene name
BCL11B (B-cell lymphoma/leukaemia 11B)
Protein description
Kruppel-like zinc finger protein. Expressed in the immune system and in the brain. Regulates thymic differentiation and survival.

Highly cited references

Pubmed IDYearTitleCitations
314815362019Programming for T-lymphocyte fates: modularity and mechanisms.29
316536912020Cell type-specific actions of Bcl11b in early T-lineage and group 2 innate lymphoid cells.25
327195212020Distinct and temporary-restricted epigenetic mechanisms regulate human αβ and γδ T cell development.19
335050232021AIM2 in regulatory T cells restrains autoimmune diseases.18
334322272021RORα is a critical checkpoint for T cell and ILC2 commitment in the embryonic thymus.14
316308912019Genetic Disorders in Prenatal Onset Syndromic Short Stature Identified by Exome Sequencing.13
33876209202114q32 rearrangements deregulating BCL11B mark a distinct subgroup of T-lymphoid and myeloid immature acute leukemia.12
309231722019Whole genome paired-end sequencing elucidates functional and phenotypic consequences of balanced chromosomal rearrangement in patients with developmental disorders.12
341033292021Enhancer Hijacking Drives Oncogenic BCL11B Expression in Lineage-Ambiguous Stem Cell Leukemia.11
325881012021Role of cancer stem cell markers ALDH1, BCL11B, BMI-1, and CD44 in the prognosis of advanced HNSCC.11
327891642020Functional definition of a transcription factor hierarchy regulating T cell lineage commitment.11
328711052020SERGIO: A Single-Cell Expression Simulator Guided by Gene Regulatory Networks.10
322552452020Distinct Notch1 and BCL11B requirements mediate human γδ/αβ T cell development.10
317750402019IRF-7 Is a Critical Regulator of Type 2 Innate Lymphoid Cells in Allergic Airway Inflammation.10
314570802019Bcl11b prevents fatal autoimmunity by promoting Treg cell program and constraining innate lineages in Treg cells.9
337124722021The transcription factor Bcl11b promotes both canonical and adaptive NK cell differentiation.8
328689122021Exploring the stage-specific roles of Tcf-1 in T cell development and malignancy at single-cell resolution.8
318174952019Novel and Rare Fusion Transcripts Involving Transcription Factors and Tumor Suppressor Genes in Acute Myeloid Leukemia.8
314794312019Chimeric antigen receptor-induced BCL11B suppression propagates NK-like cell development.8
345012392021Biologic and Therapeutic Implications of Genomic Alterations in Acute Lymphoblastic Leukemia.7
344402922021T-Cell Acute Lymphoblastic Leukemia: Biomarkers and Their Clinical Usefulness.7
323128212020Differential timing of a conserved transcriptional network underlies divergent cortical projection routes across mammalian brain evolution.7
313508252019Uncovering the Genomic Landscape in Newly Diagnosed and Relapsed Pediatric Cytogenetically Normal FLT3-ITD AML.7
314570812019Bcl11b prevents catastrophic autoimmunity by controlling multiple aspects of a regulatory T cell gene expression program.7
339361122021Epigenetic Dynamics in the Function of T-Lineage Regulatory Factor Bcl11b.6
337613492021Tunable, division-independent control of gene activation timing by a polycomb switch.6
320990382020T(6;14)(q25;q32) involves BCL11B and is highly associated with mixed-phenotype acute leukemia, T/myeloid.6
313472962019A De Novo heterozygous frameshift mutation identified in BCL11B causes neurodevelopmental disorder by whole exome sequencing.6
310673162019A de novo substitution in BCL11B leads to loss of interaction with transcriptional complexes and craniosynostosis.6
333765502021miR-21-5p promotes cell proliferation by targeting BCL11B in Thp-1 cells.5
345595522021Human cytomegalovirus expands a CD8+ T cell population with loss of BCL11B expression and gain of NK cell identity.4
337257692021Human Adipose-derived mesenchymal stem cells promote lymphocyte apoptosis and alleviate atherosclerosis via miR-125b-1-3p/BCL11B signal axis.4
334401622021Multi-scale Dynamical Modeling of T Cell Development from an Early Thymic Progenitor State to Lineage Commitment.4
333936282021Integrative analysis of miRNA-mRNA network in high altitude retinopathy by bioinformatics analysis.4
322031922021The transcription factor RelB restrains group 2 innate lymphoid cells and type 2 immune pathology in vivo.4
331685832020Endocannabinoid signalling in stem cells and cerebral organoids drives differentiation to deep layer projection neurons via CB1 receptors.4
323221902020Bcl11 Transcription Factors Regulate Cortical Development and Function.4
341122642021Lower BCL11B expression is associated with adverse clinical outcome for patients with myelodysplastic syndrome.3
338701282021BCL11B is positioned upstream of PLZF and RORγt to control thymic development of mucosal-associated invariant T cells and MAIT17 program.3
328526602021Gene expression in regenerating and scarring tails of lizard evidences three main key genes (wnt2b, egfl6, and arhgap28) activated during the regulated process of tail regeneration.3
330934452020BCL11B suppresses tumor progression and stem cell traits in hepatocellular carcinoma by restoring p53 signaling activity.3
338648882022Molecular diagnosis of childhood immune dysregulation, polyendocrinopathy, and enteropathy, and implications for clinical management.2
339465332021The Route of Early T Cell Development: Crosstalk between Epigenetic and Transcription Factors.2
336635882021Inhibition of BCL11B induces downregulation of PTK7 and results in growth retardation and apoptosis in T-cell acute lymphoblastic leukemia.2
335307022021BCL11B Regulates Arterial Stiffness and Related Target Organ Damage.2
331948852020Mutant BCL11B in a Patient With a Neurodevelopmental Disorder and T-Cell Abnormalities.2
321643542020High Specificity of BCL11B and GLG1 for EWSR1-FLI1 and EWSR1-ERG Positive Ewing Sarcoma.2
316730692020BCL11B regulates MICA/B-mediated immune response by acting as a competitive endogenous RNA.2
314074032020Insertional oncogenesis by HPV70 revealed by multiple genomic analyses in a clinically HPV-negative cervical cancer.2
352043312022Human Transcriptome Array Analysis Identifies CDR2 as a Novel Suppressed Gene for Kawasaki Disease.1
349631322021Transcription Factors Bcl11a and Bcl11b Are Required for the Production and Differentiation of Cortical Projection Neurons.1
349466632021Easy Expression and Purification of Fluorescent N-Terminal BCL11B CCHC Zinc Finger Domain.1
348657012021Redefining the biological basis of lineage-ambiguous leukemia through genomics: BCL11B deregulation in acute leukemias of ambiguous lineage.1
346671172021Positive selection in noncoding genomic regions of vocal learning birds is associated with genes implicated in vocal learning and speech functions in humans.1
335381262021Epigenetic analysis reveals significant differential expression of miR-378C and miR-128-2-5p in a cohort of relapsed pediatric B-acute lymphoblastic leukemia cases.1
336348642021Deleterious point mutations in T-cell acute lymphoblastic leukemia: Mechanistic insights into leukemogenesis.1
342872932021Discrimination of Cancer Stem Cell Markers ALDH1A1, BCL11B, BMI-1, and CD44 in Different Tissues of HNSCC Patients.1
333631422020Bcl11b/Ctip2 in Skin, Tooth, and Craniofacial System.1
320026652020Sequential pattern of sublayer formation in the paleocortex and neocortex.1
326592952020BCL11B-related disorder in two canadian children: Expanding the clinical phenotype.1
327019352020Identification of Biomarkers for Sarcoidosis and Tuberculosis of the Lung Using Systematic and Integrated Analysis.1
318285112020Increased expression of BCL11B and its recruited chromatin remodeling factors during highly active antiretroviral therapy synergistically represses the transcription of human immunodeficiency virus type 1 and is associated with residual immune activation.1
356432262022An improved CUT&RUN method for regulation network reconstruction of low abundance transcription factor.0
360009502022Diverse mutations and structural variations contribute to Notch signaling deregulation in paediatric T-cell lymphoblastic lymphoma.0
357499512022DNA methylation-based machine learning classification distinguishes pleural mesothelioma from chronic pleuritis, pleural carcinosis, and pleomorphic lung carcinomas.0
357052522022Realization of the T Lineage Program Involves GATA-3 Induction of Bcl11b and Repression of Cdkn2b Expression.0
354215412022Oncogenic isoform switch of tumor suppressor BCL11B in adult T-cell leukemia/lymphoma.0
358640062022Expression pattern and diagnostic utility of BCL11B in mature T- and NK-cell neoplasms.0
356853612022Integrative RNA profiling of TBEV-infected neurons and astrocytes reveals potential pathogenic effectors.0
356212442022Construction of ovarian metastasis-related immune signature predicting prognosis of gastric cancer patients.0
356132742022FOXG1 sequentially orchestrates subtype specification of postmitotic cortical projection neurons.0
355943392022Single-cell deletion analyses show control of pro-T cell developmental speed and pathways by Tcf7, Spi1, Gata3, Bcl11a, Erg, and Bcl11b.0
355633222022Generation of Inducible BCL11B Knockout in TAL1/LMO1 Transgenic Mouse T Cell Leukemia/Lymphoma Model.0
347388382022Diagnostic challenge of identifying cases with recurrent t(8;14)(q24.21;q32.2) Involving BCL11B in acute leukemias of ambiguous lineage: an analysis of eight patients.0
353313352022Human induced-T-to-natural killer cells have potent anti-tumour activities.0
352624862022Pum2 and TDP-43 refine area-specific cytoarchitecture post-mitotically and modulate translation of Sox5, Bcl11b, and Rorb mRNAs in developing mouse neocortex.0
347486352022Genome-Wide Association Study and Polygenic Risk Scores of Serum DHEAS Levels in a Chilean Children Cohort.0
347267302021The histone demethylase Lsd1 regulates multiple repressive gene programs during T cell development.0
347143352021Nuclear import of BCL11B is mediated by a classical nuclear localization signal and not the Krüppel-like zinc fingers.0
349008712021Case Report and Review of the Literature: Congenital Diaphragmatic Hernia and Craniosynostosis, a Coincidence or Common Cause?0
348442662021Progressive Clinical and Neuroradiological Findings in a Child with BCL11B Missense Mutation: Expanding the Phenotypic Spectrum of Related Disorder.0
346255212021Human Cytomegalovirus Infection Promotes Expansion of a Functionally Superior Cytoplasmic CD3+ NK Cell Subset with a Bcl11b-Regulated T Cell Signature.0
348878732021A Novel Germline Heterozygous BCL11B Variant Causing Severe Atopic Disease and Immune Dysregulation.0
347073982021Gene Co-Expression Analysis Identified Preserved and Survival-Related Modules in Severe Blunt Trauma, Burns, Sepsis, and Systemic Inflammatory Response Syndrome.0
338381372021Logic and lineage impacts on functional transcription factor deployment for T-cell fate commitment.0
345733702021A Recurrent De Novo Terminal Duplication of 14q32 in Korean Siblings Associated with Developmental Delay and Intellectual Disability, Growth Retardation, Facial Dysmorphism, and Cerebral Infarction: A Case Report and Literature Review.0
344020582021B-cell lymphoma/leukaemia 11B (BCL11B) expression status helps distinguish early T-cell precursor acute lymphoblastic leukaemia/lymphoma (ETP-ALL/LBL) from other subtypes of T-cell ALL/LBL.0
346038032021Very Elevated IgE, Atopy, and Severe Infection: A Genomics-Based Diagnostic Approach to a Spectrum of Diseases.0
344732342021BCL11B, the Cerberus of human leukemia.0
337836112021Bcl11b is required for proper odorant receptor expression in the mouse septal organ.0
340198152021A eutherian-specific microRNA controls the translation of Satb2 in a model of cortical differentiation.0
340253652021Development of the Entorhinal Cortex Occurs via Parallel Lamination During Neurogenesis.0
338074842021Unveiling the N-Terminal Homodimerization of BCL11B by Hybrid Solvent Replica-Exchange Simulations.0
337124742021A natural killer's hike through epigenetic landscapes.0
331620302021Gut commensals suppress interleukin-2 production through microRNA-200/BCL11B and microRNA-200/ETS-1 axes in lamina propria leukocytes of murine large intestine.0
327292402020Genes regulated by BCL11B during T-cell development are enriched for de novo mutations found in schizophrenia patients.0
321355952020[A case report of BCL11B mutation induced neurodevelopmental disorder and literature review].0
318283022020Switch hitter: Bcl11b in T cells and ILC2s.0
317087342019MicroRNA-Based Separation of Cortico-Fugal Projection Neuron-Like Cells Derived From Embryonic Stem Cells.0
315314042019Erratum: Author Correction: Bcl11b controls odorant receptor class choice in mice.0


Pubmed IDLast YearTitleAuthors
87807431996Is t(6;14) a non-random translocation in childhood acute mixed lineage leukemia?Batanian JR et al
151042872004A novel t(6;14)(q25-q27;q32) in acute myelocytic leukemia involves the BCL11B gene.Bezrookove V et al
186566902008Acute mixed lineage leukemia and a t(6;14)(q25;q32) in two adults.Georgy M et al
2364166199014q32 translocations are associated with mixed-lineage expression in childhood acute leukemia.Hayashi Y et al
119204812002Abnormalities of chromosome bands 15q13-15 in childhood acute lymphoblastic leukemia.Heerema NA et al
23999101990Cytogenetic findings in 21 cases of peripheral T-cell lymphoma.Inwards DJ et al
161793742006Chromosomal translocations are associated with poor prognosis in chronic lymphocytic leukemia.Mayr C et al
27583951989Cytogenetics of childhood acute nonlymphocytic leukemia.Raimondi SC et al


Jean-Loup Huret

t(6;14)(q25-27;q32) BCL11B/?

Atlas Genet Cytogenet Oncol Haematol. 2008-07-01

Online version: http://atlasgeneticsoncology.org/haematological/1324/t(6;14)(q25-27;q32)

External Links