t(14;18)(q32;q21) IGH/MALT1
2008-03-01 Berthold Streubel   Affiliation1.Medical University of Vienna, Dept. of Pathology, WaehringerGuertel 18-20, A-1090 Vienna, Austria
Clinics and Pathology
Disease
t(14;18)/IGH-MALT1 was detected in MALT lymphoma first. The frequencies at which the translocation occurs vary markedly with the primary site of the disease. IGH-MALT1 rearrangements were described also in other B-NHLs such as DLBCL (Diffuse Large B-Cell Lymphoma).
Genes Involved and Proteins
Gene name
IGH (Immunoglobulin Heavy)
Location
14q32.33
Gene name
MALT1 (mucosa associated lymphoid tissue lymphoma translocation gene 1)
Location
18q21.32
Protein description
Stimulation of either the T cell antigen receptor (TCR) or B cell antigen receptor leads to stimulation of protein kinase C isoforms that phosphorylate the scaffolding protein CARMA1, which subsequently recruits both Bcl-10 and MALT1 to form what is now referred to as the CARMA1-Bcl-10-MALT1 (CBM) signalosome. Once the CBM signalosome is assembled, MALT1 functions as the effector protein and mediates activation of the IKK complex, a multi subunit kinase that phosphorylates the Ikappa B proteins, which bind to and sequester the transcription factor NF-kappaB in the cytoplasm. Phosphorylation and subsequent degradation of Ikappa B leads to the release of NF-kappaB, which then translocates to the nucleus and regulates the transcription of target genes involved in the immune response to foreign antigens.
Result of the Chromosomal Anomaly
Note
Breakpoints upstream the coding exons of MALT1 resulting in an in-frame deregulation of MALT1.
Highly cited references
| Pubmed ID | Year | Title | Citations |
|---|---|---|---|
| 26457033 | 2015 | Clinicopathologic Study of Chromosomal Aberrations in Ocular Adnexal Lymphomas of Korean Patients. | 50 |
| 23720088 | 2013 | Molecular and genomic aberrations in Chlamydophila psittaci negative ocular adnexal marginal zone lymphomas. | 43 |
| 35674932 | 2022 | Extranodal MALT Lymphoma in the Oral Cavity: A Series of Three Cases with Review of Literature. | 38 |
| 17525089 | 2007 | Clinical impact of genetic aberrations in gastric MALT lymphoma: a comprehensive analysis using interphase fluorescence in situ hybridisation. | 34 |
| 19669212 | 2008 | Extranodal marginal zone lymphoma of the dura mater with IgH/MALT1 translocation and review of literature. | 33 |
| 38031233 | 2024 | Novel identification of t(14;18)(q32;q21)/IGH::MALT1 in chronic lymphocytic leukaemia. | 16 |
| 37693842 | 2023 | Waldenstrom's macroglobulinemia-like B cell lymphoma with MYD88 L265P mutation and t(14;18)(q32;q21) involving IGH-MALT1. | 11 |
| 20430965 | 2010 | The t(14;18)(q32;q21)/IGH-MALT1 translocation in MALT lymphomas is a CpG-type translocation, but the t(11;18)(q21;q21)/API2-MALT1 translocation in MALT lymphomas is not. | 5 |
| 15682443 | 2005 | MALT lymphoma with t(14;18)(q32;q21)/IGH-MALT1 is characterized by strong cytoplasmic MALT1 and BCL10 expression. | 0 |
| 17519619 | 2007 | Primary cutaneous marginal zone B-cell lymphoma may exhibit both the t(14;18)(q32;q21) IGH/BCL2 and the t(14;18)(q32;q21) IGH/MALT1 translocation: an indicator for clonal transformation towards higher-grade B-cell lymphoma? | 0 |
| 24236896 | 2014 | The t(14;18)(q32;q21)/IGH-MALT1 translocation in gastrointestinal extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). | 0 |
| 28288707 | 2017 | MALT lymphoma: Genetic abnormalities, immunological stimulation and molecular mechanism. | 0 |
| 27452667 | 2016 | MALT lymphoma: A paradigm of NF-κB dysregulation. | 0 |
| 14574335 | 2004 | Primary pulmonary MALT lymphomas show frequent and heterogeneous cytogenetic abnormalities, including aneuploidy and translocations involving API2 and MALT1 and IGH and MALT1. | 0 |
| 19965626 | 2010 | The t(14;18)(q32;q21)/IGH-MALT1 translocation in MALT lymphomas contains templated nucleotide insertions and a major breakpoint region similar to follicular and mantle cell lymphoma. | 0 |
| 16630178 | 2006 | t(14;18)(q32;q21) involving IGH and MALT1 is uncommon in cutaneous MALT lymphomas and primary cutaneous diffuse large B-cell lymphomas. | 0 |
| 18040143 | 2007 | MALT lymphoma : recent advances in aetiology and molecular genetics. | 0 |
| 28278699 | 2017 | MALT1 sequencing analyses in marginal zone B-cell lymphomas reveal mutations in the translocated MALT1 allele in an IGH-MALT1-positive MALT lymphoma. | 0 |
| 21261681 | 2011 | MALT lymphoma: many roads lead to nuclear factor-κb activation. | 0 |
| 14652825 | 2003 | T(14;18)(q32;q21) involving MALT1 and IGH genes in an extranodal diffuse large B-cell lymphoma. | 0 |
| 17122510 | 2006 | The incidence and anatomic site specificity of chromosomal translocations in primary extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) in North America. | 0 |
| 16830956 | 2006 | Primary cutaneous marginal zone B-cell lymphoma. | 0 |
| 17199743 | 2007 | MALT1, BCL10 and FOXP1 in salivary gland mucosa-associated lymphoid tissue lymphomas. | 0 |
| 18927281 | 2008 | Mucosa-associated lymphoid tissue lymphoma: novel translocations including rearrangements of ODZ2, JMJD2C, and CNN3. | 0 |
| 15682432 | 2005 | FISH analysis of MALT lymphoma-specific translocations and aneuploidy in primary cutaneous marginal zone lymphoma. | 0 |
| 23508748 | 2013 | No evidence of IGH-MALT1-translocation in the Ma-1 cell line--a reply. | 0 |
| 23508779 | 2013 | No evidence of IGH-MALT1 translocation in the Ma-1 cell line. | 0 |
| 18445693 | 2008 | Translocations involving the immunoglobulin heavy chain gene locus predict better survival in gastric diffuse large B-cell lymphoma. | 0 |
| 24679486 | 2014 | Chromosomal anomalies in primary cutaneous follicle center cell lymphoma do not portend a poor prognosis. | 0 |
| 23171357 | 2012 | Does the proliferation fraction help identify mature B cell lymphomas with double- and triple-hit translocations? | 0 |
| 20520640 | 2010 | Differential expression of NF-kappaB target genes in MALT lymphoma with and without chromosome translocation: insights into molecular mechanism. | 0 |
| 16804917 | 2006 | A novel fusion of the MALT1 gene and the microtubule-associated protein 4 (MAP4) gene occurs in diffuse large B-cell lymphoma. | 0 |
| 28505007 | 2017 | AL Amyloidoma of the Skin/Subcutis: Cutaneous Amyloidosis, Plasma Cell Dyscrasia or a Manifestation of Primary Cutaneous Marginal Zone Lymphoma? | 0 |
| 19094584 | 2008 | [Frequency of genetic aberrations in mucosa-associated lymphoid tissue lymphoma of different sites]. | 0 |
| 19576081 | 2009 | [A clinical study of chromosome translocations in extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue in Chinese patients]. | 0 |
| 20830719 | 2011 | BCL6 gene rearrangement and protein expression are associated with large cell presentation of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. | 0 |
| 19159526 | 2008 | [Primary ocular adnexal lymphoproliferative lesions: clinicopathologic features and genetic alterations]. | 0 |
| 21837708 | 2011 | Establishment of a novel MALT lymphoma cell line, ma-1, from a patient with t(14;18)(q32;q21)-positive Helicobacter pylori-independent gastric MALT lymphoma. | 0 |
| 21055028 | 2010 | [Study on genetic aberrations of ocular mucosa-associated lymphoid tissue lymphomas occurring in southern China]. | 0 |
| 27873317 | 2017 | The B-cell-activating factor signalling pathway is associated with Helicobacter pylori independence in gastric mucosa-associated lymphoid tissue lymphoma without t(11;18)(q21;q21). | 0 |
| 22382892 | 2012 | Chlamydia psittaci-negative ocular adnexal marginal zone B-cell lymphomas have biased VH4-34 immunoglobulin gene expression and proliferate in a distinct inflammatory environment. | 0 |
| 15867234 | 2005 | High relapse rate in patients with MALT lymphoma warrants lifelong follow-up. | 0 |
| 30626796 | 2018 | [MALT lymphoma accompanied by elevated serum IgM levels mimicking Waldenström's macroglobulinemia]. | 0 |
| 22781798 | 2012 | [Clinicopathologic analysis of nodal marginal zone B cell lymphoma]. | 0 |
Article Bibliography
| Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|
| 16155022 | 2005 | Molecular pathogenesis of mucosa-associated lymphoid tissue lymphoma. | Farinha P et al |
| 15286744 | 2004 | MALT lymphoma: from morphology to molecules. | Isaacson PG et al |
| 12560219 | 2003 | MALT1 is deregulated by both chromosomal translocation and amplification in B-cell non-Hodgkin lymphoma. | Sanchez-Izquierdo D et al |
| 17098202 | 2006 | Antigen-receptor signaling to nuclear factor kappa B. | Schulze-Luehrmann J et al |
| 15356642 | 2004 | Variable frequencies of MALT lymphoma-associated genetic aberrations in MALT lymphomas of different sites. | Streubel B et al |
| 11090634 | 2000 | Identification of paracaspases and metacaspases: two ancient families of caspase-like proteins, one of which plays a key role in MALT lymphoma. | Uren AG et al |
Summary

t(14;18)(q32;q21) with additional cytogenetic abnormalities in a hepatic MALT lymphoma. FISH demonstrated an IGH-MALT1 rearrangement.
Citation
Berthold Streubel
t(14;18)(q32;q21) IGH/MALT1
Atlas Genet Cytogenet Oncol Haematol. 2008-03-01
Online version: http://atlasgeneticsoncology.org/haematological/1350/t(14;18)(q32;q21)-igh-malt1
