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.
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)
The most prevalent form of MZL, accounting for about 2/3 of all cases. MALT most commonly grows in the stomach.6
The rarest type of MZL (<10% of all cases), which develops in the lymph nodes.4
A rare form of MZL (~20% of all cases),4 in which cancerous cells build up in the spleen, bone marrow or blood.7
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:
Persistent indigestions or heartburn, stomach aches, nausea and loss of appetite8 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.
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
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
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
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
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.
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.
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.
Janssen has been researching lymphoma for decades with the ultimate goal to turn blood cancer not only into a treatable but a curable condition.
In collaboration with committed cancer researchers, specialists and patient organisations, we are determined to improve and extend the quality of life of patients, and ultimately make cancer a thing of the past.
This website is developed exclusively by Janssen Pharmaceutica NV. Please note that the patient advocacy groups, and external sources listed below are an additional and independent source of information you might find useful. These groups and sources were not involved in the creation of this website and do not endorse its content in any way.
Each September, organisations all over the world that are dedicated to different types of blood cancer, including MCL, launch campaigns to raise awareness about the disease and its many forms.
The Lymphoma Coalition is a global network of lymphoma patient groups, created to spread awareness about the condition and provide a community of support for groups worldwide.
The Lymphoma Association is a charity dedicated to Lymphoma which provides in-depth information and personal patient experiences.