extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (EMZL), also known as MALT lymphoma, is a type of indolent non-Hodgkin lymphoma that arises in the lining of various organs and tissues outside the lymph nodes.

Characteristics:

• EMZL is a slow-growing cancer that typically occurs in the stomach, small intestine, salivary gland, thyroid, eyes, and lungs.
• It is composed of small B cells with different morphology that invade multiple organs.
• The disease generally follows an indolent clinical course, meaning it grows slowly and may

Extranodal marginal zone lymphoma (EMZL) of mucosa-associated lymphoid tissue (MALT), also known as MALT lymphoma, is a rare type of non-Hodgkin lymphoma. The symptoms and signs of this condition can vary depending on the location of the lymphoma.

Common Symptoms:

• Night sweats [4]
• Unexplained weight loss [4]
• Abdominal pain [6]
• Bloating [6]
• Heartburn [6]
• Acid reflux [6]

These symptoms are often similar to those experienced during an upset stomach and can be a sign of gastric MALT lymphoma. However, the location of the lymphoma can affect the severity and type of symptoms.

Other Possible Symptoms:

• Depending on the location of the lymphoma, other symptoms may include:
  • Conjunctival redness or swelling (if the lymphoma is in the eye) [8]
  • Swollen lymph nodes in the affected area
  • Fever

It's essential to note that some people with MALT lymphoma may not experience any noticeable symptoms at all. The condition can be asymptomatic, especially in its early stages.

References:

• [4] Symptoms vary based on the lymphoma location.
• [6] For gastric MALT lymphoma, symptoms often mimic those of an upset stomach and can include abdominal pain; bloating; heartburn; acid reflux.
• [8] These slides show conjunctival extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (also known as MALT lymphoma).

Diagnostic Tests for Extranodal Marginal Zone Lymphoma (MALT) of Mucosa-Associated Lymphoid Tissue

Diagnosing extranodal marginal zone lymphoma (MALT) of mucosa-associated lymphoid tissue requires a combination of clinical evaluation, imaging studies, and histopathological examination. Here are the diagnostic tests used to diagnose MALT lymphoma:

• Biopsy: A biopsy is the most common test for diagnosing MALT lymphoma [9]. A doctor will take a sample of tissue from the affected area, which is then examined under a microscope.
• Immunohistochemical evaluation: The biopsy sample is also evaluated using immunohistochemistry to identify specific markers on the cells. In MALT lymphoma, the cells are typically positive for B-cell markers CD19, CD20, and CD22, and negative for CD5, CD10, and CD23 [1].
• Radiologic imaging studies: Imaging studies such as CT scans, MRI, or PET scans may be used to evaluate the extent of disease and identify any lymph node involvement.
• Blood tests: A complete blood count (CBC) and blood chemistries may provide important information about the tissues and organs affected by MALTomas [7].
• Endoscopy and biopsy: For gastric MALT lymphoma, endoscopy with biopsy is a common diagnostic procedure. This involves inserting an endoscope into the stomach to visualize any abnormal tissue, which can then be biopsied.
• Bronchoscopy and biopsy: For lung MALT lymphoma, bronchoscopy with biopsy may be used to diagnose the condition.

References:

[1] A Di Rocco (2022) - On immunophenotypic analysis, the cells are positive for B-cell markers CD19, CD20, and CD22 and negative for CD5, CD10, and CD23. [7] U Farooq (2023) - Biopsies for gastric MALTomas are typically obtained through endoscopy, while lung MALTomas are biopsied using bronchoscopy. [9] The most common test for diagnosing mucosa-associated lymphoid tissue (MALT) lymphoma is a biopsy.

Treatment Options for Extranodal Marginal Zone Lymphoma (EMZL) of Mucosa-Associated Lymphoid Tissue

Extranodal marginal zone lymphoma (EMZL) of mucosa-associated lymphoid tissue (MALT) is a type of non-Hodgkin lymphoma that can be challenging to treat. While there is no standard chemotherapy for advanced stage EMZL, various treatment options are available depending on the stage and location of the disease.

• H. pylori eradication: For gastric MALTomas, treatment with a proton pump inhibitor (PPI) and antibiotics to eradicate H. pylori is the most important modality used in therapy [5]. This approach has been shown to be effective in inducing remission in patients with early-stage gastric EMZL.
• Chemotherapy: Chemotherapy regimens such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or bendamustine plus rituximab may be used to treat recurrent MALT lymphoma [6]. Oral drugs like fludarabine-containing regimens have also demonstrated efficacy in treating EMZL, including extranodal MALT lymphomas with advanced disease [7].
• MCP regimen: The MCP regimen (mitoxantrone, chlorambucil, and prednisone) has been shown to be effective and well-tolerated for treatment of patients with MALT lymphoma irrespective of localization [8].
• Targeted therapy and immunotherapy: For some patients, targeted therapy and immunotherapy drugs may be used as part of the treatment plan. The specific treatment approach depends on the type and stage of the disease [9].

It's essential to note that each patient's situation is unique, and a personalized treatment plan should be developed in consultation with a healthcare professional.

References:

[5] Apr 11, 2024 - Treatment depends on the type and stage of your NHL. Common treatments include chemotherapy, targeted and immunotherapy drugs, radiotherapy...

[6] Apr 11, 2024 - Your treatment depends on the type and stage of your NHL. Common treatments include chemotherapy, targeted and immunotherapy drugs, radiotherapy...

[7] The non-Hodgkin lymphoma subtype of marginal zone lymphoma represents a group of lymphomas that have been historically classified together because they appear to arise from post-germinal center marginal zone B cells.

[8] Drug use disorders are associated with significant costs to society due to lost productivity, premature mortality, increased health care expenditure, and costs related to criminal justice, social welfare, and other social consequences. About 296 million people aged 15-64 had used psychoactive drugs in 2021 and about 39.5 million people are...

[9] A drug is a chemical substance that produces a biological effect when administered to a living organism. Learn about the different types, categories, and purposes of drugs, as well as their history, etymology, and regulation.

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Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is a type of non-Hodgkin lymphoma that can be challenging to diagnose due to its similarity in presentation with other conditions. The differential diagnosis for MALT lymphoma includes:

• Helicobacter pylori infection: This bacterial infection is often associated with gastric MALT lymphoma and can cause symptoms such as epigastric pain, anorexia, and weight loss [8].
• Diffuse large B-cell lymphoma (DLBCL): DLBCL is a more aggressive type of non-Hodgkin lymphoma that can present with similar symptoms to MALT lymphoma, including fever, night sweats, and weight loss [11].
• Non-Hodgkin lymphoma (NHL): NHL is a broad category of blood cancers that includes MALT lymphoma. Other types of NHL