Bay Biosciences provides high quality, clinical grade, matched cryogenically preserved K2EDTA plasma, sera (serum) and peripheral blood mononuclear cells (PBMC), bio-fluid samples from marginal zone lymphoma (MZL) patients.
The K2EDTA plasma, sera (serum) and PBMC bio-fluid specimens are processed from marginal zone lymphoma (MZL) patient’s peripheral whole-blood using customized collection and processing protocols.
Marginal Zone Lymphoma (MZL) Overview
Marginal zone lymphoma (MZL) is a type of indolent or slow growing non-Hodgkin’s lymphoma. There are three types of MZLs, and the treatment depends on which type a patient has developed.
Lymphomas are considered either Hodgkin’s lymphoma or non-Hodgkin lymphoma. There are two main types of lymphocytes, T cells and B cells. All types of marginal zone lymphomas affect the B cells.
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 patient’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.
According to the American Cancer Society (ACS), marginal zone lymphoma (MZL) is a type of non-Hodgkin lymphoma that accounts for approximately 5–10%Trusted Source of all lymphoma cases.
Types of Marginal Zone Lymphoma (MZL)
Following are three main types of marginal zone lymphomas (MZL):
Extra nodal marginal zone B-cell lymphoma: Doctors may also refer to this as mucosa-associated lymphatic (MALT) tissue. This is the most common type of MZL. Following 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.
MALT is the most common form of MZL. It can develop in the stomach (gastric) or outside the stomach (non-gastric). It can affect different parts of the body, such as the:
- Eyes
- Lungs
- Small intestines
- Salivary glands
- Thyroid
According to the Lymphoma Research Foundation, this type accounts for 9 percent of B-cell lymphomas.
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.
This is the rarest form of the disease. It develops in the spleen, bone marrow, or both. According to the American Society of Hematology’s journal Blood, it’s present in less than 2 percent all lymphomas, and has been linked 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.
This rare type of lymphoma develops in the lymph nodes. It accounts for less than 2 percent of all MZL, according to the Lymphoma Association.
Diagnosing and treating all three types of marginal zone lymphomas is possible but can be challenging, depending on the stages of each disease.
Stages of Marginal Zone Lymphoma (MZL)
Stages of MZL include the following:
- 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.
Causes of Marginal Zone Lymphoma (MZL)
The exact cause of nodal and splenic MZL is unknown. In the case of MALT, inflammation due to an infection may be responsible.
Although it’s sometimes linked to an infection, MZL isn’t contagious. It’s also not inherited. Certain factors, however, can increase the risk of developing these types of lymphoma. Risk factors include:
- Being 65 years old or older
- History of a weakened immune system
According to researchers, MZL may develop due to autoimmune conditions, such as Sjögren’s syndrome and chronic autoimmune thyroiditis. Autoimmune conditions occur when the body mistakes its tissues as an enemy and attacks them.
Scientists 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 virus, 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 ACS states that the following may be risk factors for MZL:
- Age, as most cases occur in those over the age of 60
- Exposure to radiation therapy, chemotherapy drugs, herbicides, and insecticides
- family history, or genetics, associated with marginal zone lymphoma
The Lymphoma Research Foundation (LRF) notes that MZL is slightly more common in females.
Signs and Symptoms of Marginal Zone Lymphoma (MZL)
Symptoms of MZL vary, depending on the type. Symptoms associated with all forms of the disease include:
- Abdominal Pain
- Chest pain
- Fever without an infection
- Night Sweats
- Skin rash
- Tiredness
- Unexplained weight loss
You may also have symptoms specific to the type of lymphoma. For example, people with MALT may experience:
- Indigestion
- Nausea
- Stomach pain
- Vomiting
Nodal MZL can cause a painless lump in the groin, armpit, or neck area.
Splenic MZL can cause an abnormal blood count, fatigue, and discomfort due to an enlarged spleen.
Diagnosis of Marginal Zone Lymphoma (MZL)
To diagnose MZL, a doctor will need to stage the disease. Staging is also how your doctor decides the right treatment. It involves evaluating the location and size of the tumors and determining whether cancer has spread to other parts of the body.
Your doctor will use imaging tests to capture pictures of inside your body to stage MZL. These imaging tests include X-rays, ultrasound, computerized tomography (CT) scan and magnetic resonance imaging (MRI).
The National Cancer Institute (NCI) reports the diagnosis process begins with an extensive evaluation that includes medical history, lab tests, scans, and biopsy, when possible.
A healthcare professional may order the following tests:
- A complete blood counts.
- Blood chemistry studies
- Bone marrow aspirate and biopsy
- LDH test, to measure the amount of lactic dehydrogenase in the blood
- lymph node biopsy
- Hepatitis B or C test
- HIV test
- PET scans
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.
Treatment of Marginal Zone Lymphoma (MZL)
According to the LRF, treatment for MZL depends on the cancer’s type, stage, and location. It also depends on the patients age and overall health.
Gastric MALT
A doctor 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. The patient 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, the patient may require:
- Additional antibiotic treatment
- Radiation therapy
- Immunotherapy, either alone or along with chemotherapy
Non-Gastric MALT
Treatment will depend on the location of the cancer. For example, if the cancer is in the eye, the patient will require radiation therapy alongside antibiotics.
A doctor may also recommend watchful waiting. If patients begin developing symptoms, they will then prescribe radiation therapy for localized cases or surgery.
If it is advanced, the patient may require immunotherapy, with or without chemotherapy.
Nodal MZL
A doctor 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, patients may undergo surgery, chemotherapy, and immunotherapy.
If the hepatitis C virus is the cause, treating the infection can help to treat the lymphoma.
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