MALT lymphoma
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Mucosa associated lymphoid tissue (MALT) lymphoma, also called extranodal marginal zone B-cell lymphoma, is a type of low-grade extranodal lymphoma.
Epidemiology
MALT lymphoma represents ~7.5% of non-Hodgkin lymphomas. Average age of presentation is 60 years with a slight female predominance 1. Prevalence is estimated at 2 per 100,000 7.
Clinical presentation
Clinical presentation depends on the affected organ. B-type symptoms are rare 1,3.
Pathology
MALT lymphomas arise in epithelial tissues where lymphoid cells are not usually found. Chronic infection/inflammation has been implicated in the pathogenesis, for example Helicobacter pylori infection with gastric MALT lymphoma, and Sjogren syndrome with salivary gland MALT lymphoma 1,3. Less than 10% transform from low-grade to high-grade disease 4.
MALT lymphoma can localise throughout the entire body 1,4:
- stomach: most common; 33-50% (see: gastric lymphoma)
- associated with H. pylori infection in 90% of cases 7
- intestine: ~5%
- possible association with Coeliac disease6
- salivary glands
- associated with Sjogren syndrome
- skin
- thyroid
- associated with Hashimoto thyroiditis5
- orbit (see: orbital lymphoma)
- breast
- lung and upper airways (see: BALT lymphoma)
- kidney
- liver
- rectum
- prostate
Metastases to other sites including lymph nodes and bone marrow are not uncommon.
Radiographic features
Imaging features of MALT lymphoma depend on which organ is affected.
Treatment and prognosis
MALT lymphoma is considered an indolent disease with a good prognosis. Treatment should be tailored to the affected organ and may consistent of surgery, chemotherapy and/or radiation therapy 2. Antibiotics are used to treat gastric MALT lymphoma; there is a lack of evidence for its use in treating non-gastric MALT lymphoma 7.
-<p><strong>Mucosa associated lymphoid tissue </strong>(<strong>MALT</strong>) <strong>lymphoma</strong>, also called <strong>extranodal marginal zone B-cell lymphoma</strong>, is a type of low-grade extranodal <a href="/articles/lymphoma">lymphoma</a>. </p><h4>Epidemiology</h4><p>MALT lymphoma represents ~7.5% of <a href="/articles/non-hodgkin-lymphoma">non-Hodgkin lymphomas</a>. Average age of presentation is 60 years with a slight female predominance <sup>1</sup>. </p><h4>Clinical presentation</h4><p>Clinical presentation depends on the affected organ. B-type symptoms are rare <sup>1,3</sup>.</p><h4>Pathology</h4><p>MALT lymphomas arise in epithelial tissues where lymphoid cells are not usually found. Chronic infection/inflammation has been implicated in the pathogenesis, for example <em>Helicobacter pylori</em> infection with <a href="/articles/gastric-malt-lymphoma">gastric MALT lymphoma</a>, and <a href="/articles/sjogren-syndrome-1">Sjogren syndrome</a> with <a href="/articles/salivary-gland">salivary gland</a> MALT lymphoma <sup>1,3</sup>. Less than 10% transform from low-grade to high-grade disease <sup>4</sup>.</p><p>MALT lymphoma can localise throughout the entire body <sup>1,4</sup>:</p><ul>-<li>stomach: most common; 33% (see: <a href="/articles/gastric-lymphoma">gastric lymphoma</a>)</li>-<li>intestine: ~5%</li>-<li>salivary glands</li>- +<p><strong>Mucosa associated lymphoid tissue </strong>(<strong>MALT</strong>) <strong>lymphoma</strong>, also called <strong>extranodal marginal zone B-cell lymphoma</strong>, is a type of low-grade extranodal <a href="/articles/lymphoma">lymphoma</a>. </p><h4>Epidemiology</h4><p>MALT lymphoma represents ~7.5% of <a href="/articles/non-hodgkin-lymphoma">non-Hodgkin lymphomas</a>. Average age of presentation is 60 years with a slight female predominance <sup>1</sup>. Prevalence is estimated at 2 per 100,000 <sup>7</sup>. </p><h4>Clinical presentation</h4><p>Clinical presentation depends on the affected organ. B-type symptoms are rare <sup>1,3</sup>.</p><h4>Pathology</h4><p>MALT lymphomas arise in epithelial tissues where lymphoid cells are not usually found. Chronic infection/inflammation has been implicated in the pathogenesis, for example <em>Helicobacter pylori</em> infection with <a href="/articles/gastric-malt-lymphoma">gastric MALT lymphoma</a>, and <a href="/articles/sjogren-syndrome-1">Sjogren syndrome</a> with <a href="/articles/salivary-gland">salivary gland</a> MALT lymphoma <sup>1,3</sup>. Less than 10% transform from low-grade to high-grade disease <sup>4</sup>.</p><p>MALT lymphoma can localise throughout the entire body <sup>1,4</sup>:</p><ul>
- +<li>stomach: most common; 33-50% (see: <a href="/articles/gastric-lymphoma">gastric lymphoma</a>)<ul><li>associated with <em>H. pylori</em> infection in 90% of cases <sup>7</sup>
- +</li></ul>
- +</li>
- +<li>intestine: ~5%<ul><li>possible association with <a href="/articles/coeliac-disease-1">Coeliac disease</a> <sup>6</sup>
- +</li></ul>
- +</li>
- +<li>salivary glands<ul><li>associated with Sjogren syndrome</li></ul>
- +</li>
-<li>thyroid</li>- +<li>thyroid<ul><li>associated with <a href="/articles/hashimoto-thyroiditis">Hashimoto thyroiditis</a> <sup>5</sup>
- +</li></ul>
- +</li>
-</ul><p>Metastases to other sites including lymph nodes and bone marrow are not uncommon. </p><h4>Radiographic features</h4><p>Imaging features of MALT lymphoma depend on which organ is affected. </p><h4>Treatment and prognosis</h4><p>MALT lymphoma is considered an indolent disease with a good prognosis. Treatment should be tailored to the affected organ and may consistent of surgery, chemotherapy and/or radiation therapy <sup>2</sup>. </p>- +</ul><p>Metastases to other sites including lymph nodes and bone marrow are not uncommon. </p><h4>Radiographic features</h4><p>Imaging features of MALT lymphoma depend on which organ is affected. </p><h4>Treatment and prognosis</h4><p>MALT lymphoma is considered an indolent disease with a good prognosis. Treatment should be tailored to the affected organ and may consistent of surgery, chemotherapy and/or radiation therapy <sup>2</sup>. Antibiotics are used to treat gastric MALT lymphoma; there is a lack of evidence for its use in treating non-gastric MALT lymphoma <sup>7</sup>.</p>
References changed:
- 5. Troch M, Woehrer S, Streubel B et al. Chronic Autoimmune Thyroiditis (Hashimoto's Thyroiditis) in Patients with MALT Lymphoma. Ann Oncol. 2008;19(7):1336-9. <a href="https://doi.org/10.1093/annonc/mdn049">doi:10.1093/annonc/mdn049</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/18334510">Pubmed</a>
- 6. Smedby K, Akerman M, Hildebrand H, Glimelius B, Ekbom A, Askling J. Malignant Lymphomas in Coeliac Disease: Evidence of Increased Risks for Lymphoma Types Other Than Enteropathy-Type T Cell Lymphoma. Gut. 2005;54(1):54-9. <a href="https://doi.org/10.1136/gut.2003.032094">doi:10.1136/gut.2003.032094</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/15591504">Pubmed</a>
- 7. Kiesewetter B & Raderer M. Antibiotic Therapy in Nongastrointestinal MALT Lymphoma: A Review of the Literature. Blood. 2013;122(8):1350-7. <a href="https://doi.org/10.1182/blood-2013-02-486522">doi:10.1182/blood-2013-02-486522</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/23770778">Pubmed</a>
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