Overview
Marginal zone lymphoma (MZL) is a type of lymphoma. Lymphoma is cancer that affects the lymphatic system. The lymphatic system is made up of organs, glands, tubelike vessels and clusters of cells called lymph nodes. It's part of the body's germ-fighting immune system.
There are many types of lymphoma. Lymphoma types are often divided into two categories: Hodgkin lymphoma and non-Hodgkin lymphoma. Marginal zone lymphoma is a type of non-Hodgkin lymphoma.
Marginal zone lymphoma gets its name from the fact that the cancer starts in the marginal zone. The marginal zone is a part of the immune system where germ-fighting white blood cells live. These cells are called B cells, also known as B lymphocytes. In MZL, changes happen that turn the B lymphocytes into cancer cells. The cancer cells can grow in the lymph nodes, bone marrow, stomach, spleen and other parts of the body.
Marginal zone lymphoma is a slow-growing type of cancer. Treatment options for MZL may include treatment for infections, chemotherapy, immunotherapy, radiation therapy, surgery, targeted therapy and bone marrow transplant, also called bone marrow stem cell transplant.
Types
The types of marginal zone lymphoma (MZL) include:
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Extranodal marginal zone lymphoma (ENMZL). This type, also known as extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), is the most common type of marginal zone lymphoma. Extranodal means outside of the lymph nodes. ENMZL most commonly happens in the stomach. This is known as gastric ENMZL. It is often linked to ongoing inflammation caused by infections, especially Helicobacter pylori (H. pylori).
ENMZL can be found in other parts of the body, such as the lungs, breasts, thyroid, salivary glands or eyes. Primary cutaneous marginal zone lymphoma is a type of ENMZL found in the skin.
- Splenic marginal zone lymphoma (SMZL). This type of marginal zone lymphoma affects the spleen and may involve lymph nodes, bone marrow and blood. Some cases of SMZL have been linked to the hepatitis C virus.
- Nodal marginal zone lymphoma (NMZL). NMZL is a rare type of marginal zone lymphoma that starts in the lymph nodes.
Symptoms
Marginal zone lymphoma (MZL) signs and symptoms may depend on what part of the body is involved. Splenic MZL can cause a swollen spleen, bloating and a feeling of fullness after eating only a small amount. Nodal MZL can cause swollen lymph nodes in the neck, armpits and groin.
Extranodal MZL in the stomach can cause symptoms such as:
- Bloating.
- Nausea.
- A backwash of food or sour liquid in the throat, called reflux.
- Tenderness or pain in the belly.
- Upset stomach.
- Weight loss.
Extranodal MZL can affect other parts of the body and cause symptoms such as:
- In the salivary glands, swelling in the face and a movable tumor in the gland.
- In the structures surrounding the eye, called the ocular adnexa, a tumor, discharge, pain, discomfort, itching, dryness, double vision, and a droopy or bulging eye.
- In the lungs, cough, chest pain, shortness of breath and coughing up blood.
- In the thyroid, a growing neck tumor that can lead to difficulty swallowing, difficulty eating or drinking, and hoarseness.
- In the skin, lesions that are usually found on the belly or arms.
When to see a doctor
Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you.
Causes
It's not always clear what causes marginal zone lymphoma (MZL). Cancer happens when cells develop changes in their DNA. A cell's DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to make many more cells quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells.
Marginal zone lymphoma starts in the marginal zone, which is a part of the immune system where germ-fighting white blood cells live. These cells are called B cells, also known as B lymphocytes. The marginal zone surrounds the main areas where immune cells gather, called follicles. Marginal zone lymphoma usually grows slowly.
Some MZLs are caused by ongoing infections, such as H. pylori, hepatitis C, Chlamydophila psittaci, Campylobacter jejuni and Borrelia burgdorferi. Some MZLs are linked to autoimmune conditions such as Hashimoto disease and Sjogren syndrome.
Risk factors
Factors that can increase the risk of marginal zone lymphoma include:
- Older age. Marginal zone lymphoma can happen at any age. But it's most common in people 60 or older.
- Ongoing infections. Ongoing infections can increase the risk of developing marginal zone lymphoma. These include H. pylori, hepatitis C, Campylobacter jejuni, Chlamydophila psittaci and Borrelia burgdorferi.
- Autoimmune conditions. Having a condition that affects the immune system can increase your risk of MZL. Conditions may include Hashimoto disease and Sjogren syndrome.
Complications
A complication of marginal zone lymphoma is the risk of the cancer turning into a more aggressive type, such as diffuse large B-cell lymphoma. Marginal zone lymphoma also has a risk of coming back after treatment, known as a relapse. Repeated relapses are common.