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Marginal Zone Lymphoma (MZL)

Marginal Zone Lymphoma (MZL)

Imagine being diagnosed with not only cancer, but a very rare type of cancer that many people have never even heard of. A Marginal Zone Lymphoma (MZL) diagnosis can understandably feel overwhelming and create feelings of uncertainty about your future. Informing yourself about MZL and finding the treatment plan that suits your needs can make a significant difference in coping with the condition.

What is Marginal Zone Lymphoma (MZL)?

MZL is a slow-growing form of Non-Hodgkin’s Lymphoma, a term used for different subtypes of blood cancer. In lymphoma, the lymphocytes, which are a type of white blood cell that protect our body from infection, become affected.[1] When this happens, the blood cells start to develop abnormally and grow out of control, affecting the immune system and increasing the risk of bacterial infection.[2] In MZL, the cancer develops in marginal zones of lymphatic tissue – present in bone marrow, lymph nodes, the spleen and other organs – that are key parts of our immune system.[3] MZL can be divided into three subtypes,[4] depending on where the cancer develops:[5]


Mucosa-Associated Lymphoid Tissue (MALT)

Nodal Marginal Zone B-cell Lymphoma (NMZL)

Splenic Marginal Zone B-cell Lymphoma (SMZL)

Mucosa-Associated Lymphoid Tissue (MALT)

The most prevalent form of MZL, accounting for about 2/3 of all cases. MALT most commonly grows in the stomach.[6]

Nodal Marginal Zone B-cell Lymphoma (NMZL)

The rarest type of MZL (<10% of all cases), which develops in the lymph nodes.[4]

Splenic Marginal Zone B-cell Lymphoma (SMZL)

A rare form of MZL (~20% of all cases),[4] in which cancerous cells build up in the spleen, bone marrow or blood.[7]

What are the symptoms of Marginal Zone Lymphoma (MZL)?

MZL is a slow-growing lymphoma, which makes it difficult to self-detect the condition in the early stages. Make sure to speak to your doctor as soon as you notice any changes in your health, as it could help detect MZL or other conditions early and make a significant difference in your prognosis.

Every type of MZL comes with different symptoms:

MALT

Persistent indigestions or heartburn, stomach aches, nausea and loss of appetite[8] can be signs of gastric MALT. If the cancerous cells are located in other areas, it can be difficult to self-detect your condition, as many people with non-gastric MALT are asymptomatic. Often, non-gastric MALT is found during routine check-ups or tests for other conditions.[9] Doctors will diagnose MALT by taking a biopsy from the affected area. If the cancer is located in your stomach, they will also perform an endoscopy to get a clear idea of the progression of the disease.

NMZL

Painless and hard lumps in the neck, armpit or groin that don’t go away are common indicators of NMZL due to the lymph nodes swelling.10 To reach a conclusive diagnosis, your doctor will perform a biopsy by removing part of an enlarged lymph node that will then be examined for cancerous cells.10

SMZL

As people with SMZL are largely asymptomatic, the condition is often detected during abdomen checks or blood tests. In later stages, the spleen can enlarge due to lymphoma cells building up, causing pain in the upper abdomen. An enlarged spleen can also cause blood cells to be removed from the blood, which can make you feel very tired and out of breath.[7]

To detect SMZL, your doctor will most likely conduct blood tests or a bone marrow biopsy.11


How common is Marginal Zone Lymphoma (MZL)?

Marginal Zone Lymphoma is a rare type of blood cancer, accounting for only 8% of all Non-Hodgkin’s Lymphoma cases, of which MALT is the most prevalent.12 What causes MZL is still unknown, though research has shown that all three types are most prevalent in people aged 55-65, with a slightly higher number of women affected.13

Research has also linked SMZL to long-term Hepatitis C-infections.14


Treatment and Care

Due to the rarity of MZL, clinical trials are limited compared to more common cancers, making it difficult to identify treatments with the best outcome.14

Because of the slow-growing nature of MZL, ‘watch and wait’ is often adopted for all three types. This means that doctors will monitor the progression of your condition and not prescribe treatment unless your symptoms worsen, as there is no evidence that early intervention would favour your condition.13

If treatment is needed, a team of multidisciplinary specialists will help you decide on a treatment plan based on the progression of the disease, your overall health and how your symptoms are affecting you. For all three types of MZL, your doctor could advise:[7]

  • Chemotherapy, sometimes in combination with steroids to make the therapy more effective
  • Radiotherapy
  • Targeted therapy

If you have early-stage gastric MALT, you could benefit from antibiotic treatments. MALT is often paired with Helicobacter pylori, a bacterium that causes damage to the stomach and that can be targeted with antibiotics to shrink the lymphoma.15

It’s normal to feel uncertain about deciding on the best treatment plan for you, especially when there’s no consensus on the most optimal treatment for MZL. Make sure to ask your doctor as many questions as possible, and don’t hesitate to speak to several specialists to better understand your options.


Living with Marginal Zone Lymphoma (MZL)

Due to the rare nature and limited awareness of MZL, you may feel overwhelmed and anxious when you know little about the disease and how it can affect your life. Try to learn as much as possible about MZL and your treatment plan. Feeling in control and informed can help you better cope with what’s ahead.

MZL is a slow-growing lymphoma, which means that you can enjoy a good quality of life with the condition. To support your body’s well-being, make sure to eat a balanced diet and fit in exercise if possible, and don’t underestimate the importance of self-care. MZL and its treatment can be exhausting and uncomfortable, so it’s important to listen to your body and rest when needed.

A cancer diagnosis can feel very isolating. Be open with loved ones about how MZL affects your everyday life and don’t hesitate to speak to your doctor or consider counselling if you struggle to confide in those close to you. Understandably, cancer can turn your life upside down, so be comfortable with asking for more information on services that can support with matters such as childcare and work.

What to ask your doctor?

The list below includes example questions to help start a conversation with your health care provider. There may be other relevant questions based on your symptoms, stage, and medical history that are not listed here.

  • Which type of MZL do I have and what can I expect?
  • What are my treatment options? How much time do I have to decide?
  • How long will the treatment take?
  • What are the possible side effects? How can I manage them?
  • What side effects should I tell you about right away?
  • What happens if the treatment doesn’t work?
  • How will this affect my everyday life?
  • Can I get a second opinion? Are there any specialists I can speak to?
  • Are there any clinical trials I can join? How effective are they?
  • Can you suggest a mental health professional I can see if I start to feel overwhelmed, depressed or distressed? Can I be referred to patient support groups?

Glossary

  • Indolent lymphoma: Lymphoma that grows and spreads slowly.
  • Lymph nodes: small glands that work as filters in the body for harmful substances and that contain immune cells, or lymphocytes.
  • Lymphocytes: white blood cells that are the body’s main type of immune cells.
  • Lymphoid tissue: organized structures that support immune responses.
  • Systematic treatment: treatment that affects the whole body.
  • Incidence: the proportion or rate of persons in a population who develop a condition during a particular time period.
  • Prevalence: The proportion of persons in a population who have a condition at or during a particular time period.
  • Endoscopy: the insertion of a long, thin tube into the body to observe an internal organ or tissue in detail.
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