High-grade Hepatic Mucosa-associated Lymphoid Tissue (MALT) Lymphoma Probably Transformed from the Low-grade Gastric MALT Lymphoma

BCL10 is directly involved in t(1;14)(p22;q32) of mucosa-associated lymphoid tissue (MALT) lymphoma. Wild-type BCL10 promoted apoptosis and suppressed malignant transformation in vitro, whereas truncated mutants lost the pro-apoptotic activity and exhibited gain of function enhancement of transformation. We studied 220 lymphomas for genomic BCL10 mutation by polymerase chain reaction–single-strand conformational polymorphism and DNA sequencing. Nineteen mutations were found in 13 lymphoma specimens, as follows: 8 of 120 (6.7%) mucosa-associated lymphoid tissue (MALT) lymphomas, 4 of 42 (9.5%) follicular lymphomas, and 1 of 23 (4.3%) diffuse large B-cell lymphomas. No mutations were found in 14 mantle cell lymphomas or 21 T-cell lymphomas. High-grade MALT lymphoma tended to show a slightly higher mutation frequency (2 of 25, 8%) than low-grade MALT tumor (6 of 95, 6.3%). Among low-grade gastric MALT lymphoma, mutations were found in 3 of 11 tumors that did not respond to Helicobacter pylori eradication therapy, but none were found in 22 tumors that regressed completely after H pylori eradication. All 14 potentially pathogenic mutations were distributed in the carboxyl terminal domain of BCL10. Deletion accounted for 10 of these mutations; 10 of 14 mutations caused truncated forms of BCL10. Western blot analysis of a mutant case confirmed the presence of truncated BCL10 products of anticipated size. Our results suggest that BCL10 mutation may play a pathogenic role in B-cell lymphoma development, particularly in aggressive and antibiotic unresponsive MALT lymphomas, and may further implicate the biologic importance of the carboxyl terminal of the molecule.

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Low-grade gastric mucosa-associated lymphoid tissue lymphoma: Clinicopathological factors associated with Helicobacter pylori eradication and tumor regression.

Journal of Clinical Oncology ◽

10.1200/jco.2020.38.4_suppl.353 ◽

2020 ◽

Vol 38 (4_suppl) ◽

pp. 353-353

Author(s):

Hyun Ik Shim ◽

Dong Ho Lee ◽

Jae Ho Cho ◽

Cheol Min Shin ◽

Hyuk Yoon ◽

...

Keyword(s):

Helicobacter Pylori ◽

Gastric Mucosa ◽

Malt Lymphoma ◽

Lymphoid Tissue ◽

Tumor Regression ◽

Neutrophil Infiltration ◽

Tumor Location ◽

Gastric Malt Lymphoma ◽

Low Grade ◽

H Pylori

353 Background: Eradication of Helicobacter pylori is widely accepted as the initial therapy for low-grade gastric mucosa-associated lymphoid tissue (MALT) lymphoma. The aim of this study was to assess the remission and relapse rates of low-grade gastric MALT lymphoma after H. pylori eradication and to identify the clinical factors affecting remission. Methods: We retrospectively analyzed 151 patients diagnosed with gastric MALT lymphoma from May 2003 to December 2018. Results: Of the 151 patients, 112 (74.2%) had an H. pylori infection. Total regression rates with eradication was 90.2% (101/112) in H. pylori-positive patients and 55% (11/20) in H. pylori-negative patients. Age, sex, tumor location, endoscopic findings, and the severity of mononuclear lymphocytes were not related to achieving successful initial H. pylori eradication and remission. However, patients with a smaller H. pylori burden ( p=0.030) and less neutrophil infiltration ( p=0.003) were more likely to achieve a successful initial H. pylori eradication. H. pylori ( p<0.001) and the burden ( p=0.020) were significantly related to remission of MALT lymphoma. Conclusions: The results show that H. pylori burden and neutrophil infiltration were inversely related to the success of the initial H. pylori eradication procedure and that the H. pylori burden was inversely related to the remission of MALT lymphoma.

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Mucosa-associated lymphoid tissue lymphoma arising from the kidney

Canadian Urological Association Journal ◽

10.5489/cuaj.1533 ◽

2014 ◽

Vol 8 (1-2) ◽

pp. 86 ◽

Cited By ~ 2

Author(s):

Naoya Niwa ◽

Nobuyuki Tanaka ◽

Minoru Horinaga ◽

Hiroshi Hongo ◽

Yujiro Ito ◽

...

Keyword(s):

B Cell ◽

Malt Lymphoma ◽

Burkitt Lymphoma ◽

Lymphoid Tissue ◽

Cell Lymphoma ◽

Cell Lineage ◽

B Cell Lymphoma ◽

Low Grade ◽

High Grade ◽

Renal Lymphoma

Primary renal lymphoma is rare, and most are intermediate- and high-grade lymphomas of B-cell lineage, such as diffuse large B-cellor Burkitt lymphoma. We report a case of low-grade B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) arising from the kidney. Only a few cases of primary renal MALT lymphoma have been published.

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Helicobacter pylori and mucosa-associated lymphoid tissue: what's new

Hematology ◽

10.1182/asheducation-2013.1.109 ◽

2013 ◽

Vol 2013 (1) ◽

pp. 109-117 ◽

Cited By ~ 25

Author(s):

Sung-Hsin Kuo ◽

Ann-Lii Cheng

Keyword(s):

Malt Lymphoma ◽

Lymphoid Tissue ◽

Early Stage ◽

Eradication Therapy ◽

Gastric Malt Lymphoma ◽

High Grade ◽

First Line ◽

Histological Evidence ◽

H Pylori

AbstractLow-grade mucosa-associated lymphoid tissue (MALT) lymphoma of the stomach, gastric MALT lymphoma, is associated with Helicobacter pylori infection. The eradication of H pylori using antibiotics is successful in 60% to 80% of affected patients. In contrast to the previous paradigm, we and other investigators have shown that a certain proportion of patients with H pylori–positive early-stage diffuse large B-cell lymphoma (DLBCL) of the stomach with histological evidence of MALT lymphoma, including high-grade transformed gastric MALT lymphoma and gastric DLBCL(MALT), achieved long-term complete pathological remission (pCR) after first-line H pylori eradication therapy, indicating that the loss of H pylori dependence and high-grade transformation are separate events in the progression of gastric lymphoma. In addition, patients with H pylori–positive gastric DLBCL without histological evidence of MALT (gastric pure DLBCL) may also respond to H pylori eradication therapy. A long-term follow-up study showed that patients who achieved pCR remained lymphoma free. Gastric MALT lymphoma is indirectly influenced by H pylori infection through T-cell stimulation, and recent studies have shown that H pylori–triggering chemokines and their receptors, H pylori–associated epigenetic changes, H pylori–regulated miRNA expression, and tumor infiltration by CD4+CD25+ regulatory T cells contribute to lymphomagenesis of gastric MALT lymphoma. Recent studies have also demonstrated that the translocation of CagA into B lymphocytes inhibits apoptosis through p53 accumulation, BAD phosphorylation, and the up-regulation of Bcl-2 and Bcl-XL expression. In gastric MALT lymphoma, CagA may stimulate lymphomagenesis directly, through the regulation of signal transduction, and intracellular CagA is associated with H pylori dependence. These findings represent a substantial paradigm shift compared with the classical theory of H pylori–reactive T cells contributing indirectly to the development of MALT lymphoma. In conclusion, a wide range of H pylori–related gastric lymphomas have been identified. The use of antibiotics as the sole first-line therapy for early-stage gastric pure DLBCL requires validation in a prospective study. The clinical and biological significance of the CagA oncoprotein in the lymphomagenesis of gastric MALT lymphoma warrants further study.

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Differentiation of Low-Grade Gastric MALT Lymphoma and High-Grade Gastric MALT Lymphoma: The Clinical Value of Ga-67 Citrate Scintigraphy—A Pilot Study

Cancer Investigation ◽

10.1081/cnv-120005908 ◽

2002 ◽

Vol 20 (7-8) ◽

pp. 939-943 ◽

Cited By ~ 4

Author(s):

Chang-Hu Hsu ◽

Shung-Shung Sun ◽

Chia-Hung Kao ◽

Cheng-Chieh Lin ◽

Cheng-Chun Lee

Keyword(s):

Pilot Study ◽

Malt Lymphoma ◽

Gastric Malt Lymphoma ◽

Low Grade ◽

High Grade ◽

Clinical Value

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Epstein-Barr Virus–Associated High-Grade B-Cell Lymphoma of Mucosal-Associated Lymphoid Tissue in a 9-Year-Old Boy

Archives of Pathology & Laboratory Medicine ◽

10.5858/2000-124-1520-ebvahg ◽

2000 ◽

Vol 124 (10) ◽

pp. 1520-1524 ◽

Cited By ~ 2

Author(s):

Jianguo Tao ◽

Leonard Kahn

Keyword(s):

B Cell ◽

Malt Lymphoma ◽

Lymphoid Tissue ◽

Epstein Barr Virus ◽

B Cell Lymphoma ◽

Low Grade ◽

Renal Tubules ◽

High Grade ◽

Barr Virus ◽

Epstein Barr

Abstract We report an unusual case of Epstein-Barr virus (EBV)-associated mucosal-associated lymphoid tissue (MALT) lymphoma involving the lungs, kidneys, and axillary lymph nodes in a child with congenital hypoadrenalism and panhypopituitarism. The patient presented with an aggressive clinical course and histologic evolution. Initial biopsies (1994) of the lung and kidney revealed histologic features of low-grade B-cell MALT lymphoma with lymphoepithelial lesions within the renal tubules and bronchial epithelium. Subsequent biopsies (1996, 1997, and 1999) revealed progressively greater cytologic atypia, polymorphism, and necrosis; an increased mitotic rate; and a preponderance of large cells, indicative of progression from a low-grade to a high-grade MALT lymphoma. Immunophenotyping of the lung and lymph node lesions revealed identical surface marker profiles: cells were CD19+, CD20+, immunoglobulin (Ig) G+, κ+, λ−, CD5−, CD10−, CD23−, and IgM−, and also negative for T-cell markers. Genotypic analysis demonstrated the presence of immunoglobulin heavy chain rearrangement and monoclonality of EBV in the lung lesion by Southern blot hybridization and polymerase chain reaction (PCR). The clinicopathologic features suggest that these lesions might represent an immunosupression-related continuum of low-grade to high-grade MALT lymphomas. Infection with EBV may have contributed to this tumor's aggressive clinical and histologic evolution.

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Evaluation of the Association of Nine Helicobacter pylori Virulence Factors with Strains Involved in Low-Grade Gastric Mucosa-Associated Lymphoid Tissue Lymphoma

Infection and Immunity ◽

10.1128/iai.72.2.880-888.2004 ◽

2004 ◽

Vol 72 (2) ◽

pp. 880-888 ◽

Cited By ~ 75

Author(s):

Philippe Lehours ◽

Armelle Ménard ◽

Sandrine Dupouy ◽

Bernard Bergey ◽

Fréderique Richy ◽

...

Keyword(s):

Helicobacter Pylori ◽

Gastric Mucosa ◽

Virulence Factors ◽

Malt Lymphoma ◽

Lymphoid Tissue ◽

Gastric Lymphoma ◽

Gastric Malt Lymphoma ◽

Low Grade ◽

H Pylori ◽

Few Data

ABSTRACT Helicobacter pylori has been associated with the development of two malignant diseases: gastric adenocarcinoma and gastric mucosa-associated lymphoid tissue (MALT) lymphoma. Although the cag pathogenicity island, especially the cagA gene, has been linked with adenocarcinoma, few data concerning H. pylori pathogenic factors involved in low-grade gastric MALT lymphoma are available. The goal of this study was to analyze the prevalence of and correlation between genes coding for seven H. pylori virulence factors (cagA, cagE, vacA, iceA, babA, hopQ, and oipA) and two novel adhesins (sabA and hopZ) by comparing a collection of 43 H. pylori strains isolated from patients with low-grade gastric MALT lymphoma to 39 strains isolated from age-matched patients with gastritis only. Our results show that taken individually, none of the nine genes tested can be considered associated with MALT strains and allow us to conclude that MALT pathogenesis is not linked with more proinflammatory H. pylori strains. We demonstrated that in patients infected with strains harboring the iceA1 allele, sabA functional status, and hopZ “off” status, the odds of developing a MALT lymphoma were 10 times higher. However, the low prevalence of such strains (10 of 43 MALT strains) renders this triple association a low-sensitivity marker for MALT strains. Our data confirmed that H. pylori virulence factors are correlated with one another. If the involvement of H. pylori in MALT lymphoma is well established, the pathomechanism by which gastric lymphoma occurs remains to be identified.

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