Marginal zone lymphoma (MZL) is a slow growing type of non-Hodgkin lymphoma (NHL). Often seen more in women than in men, the average age of diagnosed patients is 60 years old.
Marginal zone lymphoma accounts for about 8% of all non-Hodgkin lymphoma cases. It is differentiated between 3 similar subgroups depending on chromosomal, genetics and immunophenotypes. The three groups include nodal marginal zone lymphoma, extra-nodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT), and splenic marginal zone lymphoma.
Marginal Zone Lymphoma Subtypes
Nodal Marginal Zone Lymphoma: Occurs in lymph nodes and accounts for approximately 10% of all MZL cases. It involve lymphomas that share common peripheral and paraaortic lymphadenopathies, more frequently seen in an advanced stage due to bone marrow involvement. The clinical course is slow and similar to non-curative low-grade lymphomas.
Extra Nodal Marginal Zone Lymphoma: Occurs outside of the lymph nodes in places such as the stomach, small intestine, salivary gland, thyroid, eyes and lungs. It is the most common form of MZL and accounts for about 65% of all MZL cases per year. In many cases of MALT lymphoma, the patient has a past medical history of chronic infection, inflammation, or autoimmune disease in the affected organ.
Splenic Marginal Zone Lymphoma: Most commonly seen in the spleen and blood, it has been associated with the hepatitis C virus infection. Splenic MZL accounts for approximately 20% of MZL cases diagnosed each year. It is more commonly seen with peripheral blood and bone marrow involvement. Extra nodal and peripheral lymph nodes are rarely involved.
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What Are the Symptoms of Marginal Zone Lymphoma?
The symptoms of MZL vary depending on the type of B-cell lymphoma and how advanced it is in the body. Some of the main symptoms detected include:
- Swelling of lymph nodes in the neck, armpit, or groin area
- Chest or abdominal pain including indigestion, nausea, and vomiting
- Severe coughing
- Difficulty breathing
- High fever without infection and night sweats
- Unexplained excessive and sudden weight loss
- Skin rash
Marginal Zone Lymphoma Treatment
The method of treatment for marginal zone lymphoma depends on the type, stage, and location of the disease. It also depends on the patient’s age, overall health, and symptoms associated with lymphoma. Since gastric MALT lymphoma is often the result of infection with Helicobacter pylori, the initial treatment is usually antibiotic therapy for two weeks. To help prevent or heal ulcers, stomach acid production is reduced.
Non-gastric MALT can occur in various areas of the body. Therefore, treatment is usually planned according to the exact location and extent of spread throughout the body. Since Nodal MZL is often a slow-growing disease, doctors may adopt an active surveillance approach until symptoms occur. When treatment is indicated, options include radiation therapy, chemotherapy, and immunotherapy.
In splenic MZL, treatment is not always immediately necessary. When treatment is considered appropriate, there are several options. Splenectomy may be considered for some patients. Rituximab, a monoclonal antibody, with or without chemotherapy, can be given to patients who are not suitable for surgery.