TY - JOUR
T1 - Marginal-zone B-cell lymphoma of extranodal mucosa-associated lymphoid tissue type
T2 - Molecular genetics provides new insights into pathogenesis - Commentary
AU - Vega, Francisco
AU - Medeiros, L. Jeffrey
N1 - Copyright:
Copyright 2013 Elsevier B.V., All rights reserved.
PY - 2001/11
Y1 - 2001/11
N2 - Marginal zone B-cell lymphoma of extranodal mucosa-associated lymphoid tissue (MALT) type is recognized as a distinct clinicopathologic entity in the revised European-American lymphoma (REAL) and recently published World Health Organization (WHO) classifications. These neoplasms are thought to arise from the extranodal equivalent of the lymph node marginal zone. Two recurrent chromosomal translocations, to date, have been implicated in the pathogenesis of these neoplasms. The t(11;18)(q21;q21), which is far more common, disrupts the api2 gene on chromosome 11q21 and the malt1 (mlt) gene on chromosome 18q21, resulting in the synthesis of a novel fusion gene and protein, API2-MALT1. The t(1;14)(p22;q32), which is uncommon, juxtaposes the bcl-10 gene on chromosome 1p22 adjacent to the immunoglobulin heavy chain (IgH) gene on chromosome 14, wherein BCL10 is over-expressed via the influence of the IgH enhancer. BCL-10 may then form a complex with MALT1 in the cell. Both translocations result in increased inhibition of apoptosis, conferring a survival advantage. Recent work suggests that API2-MALT1 and BCL-10-MALT1 may activate NF-kB and a common downstream signaling pathway.
AB - Marginal zone B-cell lymphoma of extranodal mucosa-associated lymphoid tissue (MALT) type is recognized as a distinct clinicopathologic entity in the revised European-American lymphoma (REAL) and recently published World Health Organization (WHO) classifications. These neoplasms are thought to arise from the extranodal equivalent of the lymph node marginal zone. Two recurrent chromosomal translocations, to date, have been implicated in the pathogenesis of these neoplasms. The t(11;18)(q21;q21), which is far more common, disrupts the api2 gene on chromosome 11q21 and the malt1 (mlt) gene on chromosome 18q21, resulting in the synthesis of a novel fusion gene and protein, API2-MALT1. The t(1;14)(p22;q32), which is uncommon, juxtaposes the bcl-10 gene on chromosome 1p22 adjacent to the immunoglobulin heavy chain (IgH) gene on chromosome 14, wherein BCL10 is over-expressed via the influence of the IgH enhancer. BCL-10 may then form a complex with MALT1 in the cell. Both translocations result in increased inhibition of apoptosis, conferring a survival advantage. Recent work suggests that API2-MALT1 and BCL-10-MALT1 may activate NF-kB and a common downstream signaling pathway.
KW - Extranodal marginal-zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT)
KW - Molecular genetics
KW - api2
KW - malt1
KW - t(11;8)
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U2 - 10.1097/00125480-200111000-00001
DO - 10.1097/00125480-200111000-00001
M3 - Comment/debate
C2 - 11707622
AN - SCOPUS:0035515479
SN - 1072-4109
VL - 8
SP - 313
EP - 326
JO - Advances in anatomic pathology
JF - Advances in anatomic pathology
IS - 6
ER -