Marginal zone lymphoma (MZL) is a type of indolent non-Hodgkin lymphoma. This means that it is slow-growing. There are three types of MZLs, and treatment will depend on which type has developed.
The lymphatic system protects the body from viruses and bacteria. Lymph cells, vessels, fluid, and nodes work together to boost the immune system and fight infections.
Lymph fluid flows through the lymph vessels and contains white blood cells called lymphocytes. In non-Hodgkin lymphoma, the cancer begins in the person’s lymphocytes. The affected cells multiply and gather in different parts of the lymphatic system.
The lymphocytes lose their ability to fight infection, causing people to become more prone to infections.
The type of MZL depends on where the cancer cells develop in the lymphatic system.
This article discusses marginal zone lymphoma, including its types and stages, associated symptoms, and causes and risk factors. It also looks at the potential treatment options available and where a person might find support.
Lymphomas are considered either Hodgkin’s lymphoma or non-Hodgkin lymphoma.
According to the American Cancer Society (ACS), MZL is a type of non-Hodgkin lymphoma that accounts for approximately
There are two main types of lymphocytes — T cells and B cells. All types of marginal zone lymphomas affect the B cells.
There are three main types of MZL, which include:
- Extranodal marginal zone B-cell lymphoma: Healthcare professionals may also refer to this as mucosa-associated lymphatic (MALT) tissue. This is the most common type of MZL. There are two types of MALT lymphomas:
- Non-gastric MALT lymphomas begin outside the gastrointestinal tract, in areas where there are few lymphoid tissues, such as the stomach, small bowel, lungs, eye, and salivary glands. They are connected to infections with bacteria, such as Chlamydophila and Campylobacter, and viruses.
- Gastric MALT begins in the stomach and has links to infection with Helicobacter pylori, which is the bacteria that leads to stomach ulcers.
- Splenic MZL: The cancer occurs in the spleen, bone marrow, and blood. It is rare, accounting for
1–2% of non-Hodgkin lymphomas. It has links to the hepatitis C virus. - Nodal MZL: This type usually begins and stays in the lymph nodes. Nodal MZL is also rare and contains elements of both MALT and splenic MZLs. It occurs
mainly in adults but may affect children.
Diagnosing and treating all three types of marginal zone lymphomas is possible but can be challenging, depending on the stages of each disease.
The
A healthcare professional may order the following tests:
- a complete blood count
- blood chemistry studies
- LDH test, to measure the amount of lactic dehydrogenase in the blood
- hepatitis B or C test
- HIV test
- CT or PET scans
- bone marrow aspiration and biopsy
- lymph node biopsy
If these procedures confirm a diagnosis of marginal zone lymphoma, a doctor will need to diagnose its stage to verify its development and create the right treatment plan.
Stages of MZL
- Stage I: Healthcare professionals divide the first stage into either I or IE. In stage I, the cancer is in the lymph system. In stage IE, the cancer is outside the lymph system.
- Stage II: Healthcare professionals divide this stage into II and IIE. Marginal zone lymphoma stage II means the cancer is in two or more lymph nodes. Stage IIE means cancer started in the lymph nodes but has spread to areas outside the lymph system.
- Stage III: This is when the cancer is in groups of lymph nodes above or below the diaphragm or above the diaphragm and in the spleen.
- Stage IV: This is the most challenging to treat. Stage IV can mean:
- The MZL has spread to organs outside the lymph system.
- It is in two or more groups of lymph nodes either above or below the diaphragm, plus in an organ outside the lymph system.
- MZL can be in lymph nodes above and below the diaphragm and in an organ outside the lymph system.
- The cancer is in the liver, bone marrow, lung, and cerebrospinal fluid. It has not spread from the nearby lymph nodes.
If a person experiences any of the following, they should talk with a doctor about receiving further evaluation:
- swollen lymph nodes
- swollen abdomen
- excessive fatigue
- night sweats
- breathing problems
- chest pain
- flu-like symptoms
- frequent illnesses
According to
Researchers also suggest that infections that can cause cell division, overstimulate the immune system, or suppress the immune system can lead to MZL.
Infections that infect lymphocytes, interrupt their typical functions and promote cell division include Epstein-Barr, Human Herpesvirus 8, and Human T-lymphotropic virus type I.
Infections that overstimulate the immune system include:
- Hepatitis C virus
- Chlamydophila psittaci
- Borrelia burgdorferi
- Campylobacter jejuni
A virus that can suppress the immune system includes HIV.
The
- exposure to radiation, chemotherapy drugs, herbicides, and insecticides
- family history, or genetics, associated with marginal zone lymphoma
- age, as most cases occur in those over the age of 60
The Lymphoma Research Foundation (LRF) notes that MZL is slightly more common in females.
According to the LRF, treatment for MZL depends on the cancer’s type, stage, and location. It also depends on the person’s age and overall health.
Gastric MALT
A healthcare professional can treat gastric MALT lymphoma with triple therapy of two antibiotics alongside proton pump inhibitors. This is because it is usually the result of an infection with H. pylori.
A person will need to take this for 2 weeks at a time.
In 90% of cases, this is enough to cure gastric MALT, although treatment may last for months.
If the gastric MALT returns or does not respond to treatment after three attempts, a person may require:
- additional antibiotic treatment
- radiation therapy
- immunotherapy, either alone or alongside chemotherapy
Non-gastric MALT
Treatment will depend on the location of the cancer. For example, if the cancer is in the eye, a person will require radiation therapy alongside antibiotics.
A healthcare professional may also recommend watchful waiting. If people begin developing symptoms, they will then prescribe radiation therapy for localized cases or surgery.
If it is advanced, a person may require immunotherapy, with or without chemotherapy.
Nodal MZL
A healthcare professional may recommend watchful waiting until symptoms appear. Otherwise, treatment includes radiation therapy, chemotherapy, and immunotherapy.
Splenic MZL
This type of MZL may not require treatment. If it does, people may undergo surgery, chemotherapy, and immunotherapy.
If the hepatitis C virus is the cause, treating the infection can help to treat the lymphoma.
According to the Leukemia & Lymphoma Society, the 5-year survival rates are as follows:
- MALT lymphomas: 88.7%
- Splenic MZL: 79.7%
- Nodal MZL: 76.5%
The survival rate refers to the proportion of people still alive for a length of time after receiving a particular diagnosis. For example, the 5-year survival rate of 50% means that 50%, or half, of the people are still alive 5 years after receiving the diagnosis.
It is important to remember that these figures are estimates and based on previous studies or treatments. A person can consult a healthcare professional about how their condition is going to affect them.
MZL is a form of non-Hodgkin lymphoma. There are three main types, including mucosa-associated lymphoma tissue, nodal, and splenic.
The treatment options depend on the type, stage, and location of the MZL.
People should contact a healthcare professional if they experience any symptoms of MLZ, such as swollen lymph nodes.