t(11;18)(q21;q21) BIRC3/MALT1
2001-09-01 Peter Marynen  , Mathijs Baens   Affiliation1.Center for Human Genetics, Molecular Genetics, Flanders Interuniversity Institute for Biotechnology, Catholic University of Leuven, Leuven, Belgium
Clinics and Pathology
Disease
B-cell non Hodgkin lymphoma (NHL), marginal zone B-cell lymphoma of Mucosa-Associated Lymphoid Tissue ( ) -type
Phenotype stem cell origin
Marginal zone B-cells
Epidemiology
found in extranodal MZBCL or MALT-type (50 %), absent in splenic and nodal MZBCL
Prognosis
For gastric MALT-type lymphomas, t(11;18) is a clonal marker for resistance to Helicobacter pylori eradication therapy and antigen independent growth
Cytogenetics

t(11;18) FISH - Baens Mathijs, Peter Marynen
Genes Involved and Proteins
Gene name
BIRC3 (baculoviral IAP repeat-containing 3)
Location
11q22.2
Protein description
68 kDa; 604 amino acids. Member of the inhibitor of apoptosis (IAP) protein family.
Gene name
MALT1 (mucosa associated lymphoid tissue lymphoma translocation gene 1)
Location
18q21.32
Protein description
92 kDa; 824 amino acids
Result of the Chromosomal Anomaly
Description
5 API2 on chromosome 11q21 translocated on chromosome 18 in frame with 3 MALT1. Deletions often exclude the expression of the reciprocal 5 MLT API2 3 transcript.

BIRC3/MALT1 fusion protein - Baens Mathijs, Peter Marynen
Description
N- term API2-MLT C-term fusion protein; All MALT-type lymphomas reported with a t(11;18) express an in frame API2-MLT fusion protein with consistently the three BIR domains of API2 fused to the caspace p20 domain and VDJ4-like domain of MLT.
Highly cited references
| Pubmed ID | Year | Title | Citations |
|---|---|---|---|
| 34463316 | 2022 | Clinicopathologic Findings in Patients With Initial Diagnosis of Extranodal Marginal Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT) in Colorectal Mucosa. | 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 |
| 22890838 | 2013 | High-resolution genomic profiling reveals clonal evolution and competition in gastrointestinal marginal zone B-cell lymphoma and its large cell variant. | 0 |
| 31631721 | 2020 | Primary Low-Grade B-Cell Lymphoma of Skull With Translocation Between Immunoglobulin and Interferon Regulatory Factor 4 Genes. | 0 |
Article Bibliography
| Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|
| 10523859 | 1999 | A novel gene, MALT1 at 18q21, is involved in t(11;18) (q21;q21) found in low-grade B-cell lymphoma of mucosa-associated lymphoid tissue. | Akagi T et al |
| 10751367 | 2000 | The product of the t(11;18), an API2-MLT fusion, marks nearly half of gastric MALT type lymphomas without large cell proliferation. | Baens M et al |
| 11066071 | 2000 | Structure of the MLT gene and molecular characterization of the genomic breakpoint junctions in the t(11;18)(q21;q21) of marginal zone B-cell lymphomas of MALT type. | Baens M et al |
| 10979968 | 2000 | Detection of t(11;18)(q21;q21) by interphase fluorescence in situ hybridization using API2 and MLT specific probes. | Dierlamm J et al |
| 10907943 | 2000 | The product of the t(11;18), an API2-MLT fusion, is an almost exclusive finding in marginal zone cell lymphoma of extranodal MALT-type. | Maes B et al |
Summary
Fusion gene
BIRC3/MALT1 BIRC3 (11q22.2) MALT1 (18q21.32) M|BIRC3/MALT1 BIRC3 (11q22.2) MALT1 (18q21.32) M t(11;12;18)(q22;q13;q21) t(11;14;18)(q22;q32;q21) t(11;18)(q22;q21) t(14;18)(q32;q21) t(6;18;11)(q24;q21;q22)|BIRC3/MALT1 BIRC3 (11q22.2) MALT1 (18q21.32) TIC

t(11;18)(q21;q21) BIRC3/MALT1 Diagram and G-banding - Courtesy Charles Bangs; R-banded karyotype - Courtesy Karolien Beel, Peter Meeus, Geneviu00e8ve Ameye and Lucienne Michaux
Citation
Peter Marynen ; Mathijs Baens
t(11;18)(q21;q21) BIRC3/MALT1
Atlas Genet Cytogenet Oncol Haematol. 2001-09-01
Online version: http://atlasgeneticsoncology.org/haematological/2022
