Mantle Cell Lymphoma: Symptoms, Causes, Risks, Treatment

Mantle cell lymphoma (MCL) is an aggressive type of non-Hodgkin lymphoma that originates from B-cells, a type of white blood cell (WBC). Mantle cell lymphoma prevalence is around 1 in 2,00,000. 

In India, 6% of all newly diagnosed non-Hodgkin lymphoma cases are mantle cell lymphoma. Despite being relatively uncommon, MCL presents significant clinical complexities, requiring specialized management and treatment approaches.

The unique features of mantle cell lymphoma make it a topic of extensive research and investigation in the field of oncology. While there is no cure for the disease, there are treatment options to put the condition into remission. 

The following blog discusses mantle cell lymphoma pathology outlines, markers, symptoms, treatment, etc. Continue reading to find out. 

Mantle Cell Lymphoma, the subtype of non-Hodgkin lymphoma, is a blood cancer primarily affecting the lymphatic system. It arises from abnormal B-cells, a type of white blood cell responsible for producing antibodies to fight infections. MCL gets its name from the mantle zone, a region in the lymph nodes where these abnormal B-cells are located.

MCL is characterised by its aggressive behaviour, which grows and spreads rapidly to various organs and tissues, including the bone marrow, bloodstream, and digestive system. It is often diagnosed at an advanced stage when the cancer has spread throughout the body. This makes treatment more challenging.

Mantle cell lymphoma is primarily classified into two major subtypes. Within these subtypes, there can be variations in clinical presentation, behaviour, and treatment response. These subtypes include:

1. Classical Mantle Cell Lymphoma: It is the most common type of MCL that affects lymph nodes and extranodal sites (outside the lymph nodes). Classical mantle cell lymphoma is usually a fast-growing cancer with two variants: 

1. Blastoid mantle cell lymphoma (a very aggressive subtype of MCL that primarily affects lymph nodes, bone marrow, and spleen) 

2. Pleomorphic mantle cell lymphoma (an aggressive subtype that affects the head and neck)

2. Leukaemic Non-nodal Mantle Cell Lymphoma: This subtype is less common. It tends to have an indolent (slow-growing) course and involves the bone marrow, spleen, and peripheral blood. 

Mantle Cell Lymphoma Stages

Mantle cell lymphoma stages range from Stage I to Stage IV. Stages of MCL help determine the extent of the disease and guide treatment decisions. These stages include:

1. Stage I: It involves cancer localized to a single lymph node region or a group of lymph nodes next to each other. 

2. Stage II: Cancer is in two or more lymph node areas on the same side of the diaphragm or groups of lymph nodes next to each other. 

3. Stage III: Lymphoma is present in lymph nodes on both sides of the diaphragm or nodes above the diaphragm involving the spleen. 

4. Stage IV: Includes the spread of cancer to distant tissues or organs beyond the lymphatic system. 

For those wondering what are the symptoms of mantle cell lymphoma, the disease can present with various symptoms. However, some individuals with MCL may not experience any noticeable symptoms, especially in the early stages of the disease. This leads to a delay in diagnosis. When mantle cell lymphoma symptoms do occur, they can vary in severity and may include:

1. Swollen Lymph Nodes: The most common MCL symptom is the presence of swollen lymph nodes, particularly in the neck, armpit, or groyne. It occurs due to the abnormal growth and accumulation of lymphoma cells within the lymph nodes.

2. Nausea and Vomiting: Mantle cell lymphoma in the stomach can cause swelling of the stomach lining, leading to nausea or vomiting.

3. Fatigue: Many individuals with MCL may experience persistent fatigue, even with adequate rest. Fatigue may result from anaemia (low red blood cell count) caused by cancer cells in the bone marrow. 

4. A Sense of Fullness or Discomfort: MCL can involve the enlargement of tonsils, liver, or spleen. This may lead to discomfort or a feeling of fullness.

5. Gastrointestinal problems: MCL can cause lymphoma cell infiltration in the gastrointestinal tract. This may cause symptoms like abdominal pain, diarrhoea, indigestion, or bloating. 

6. Pressure or pain in the lower back: When MCL spreads to bone marrow or lymph nodes in the lower back, it can result in compression on nearby structures. This leads to pain or pressure in the lower back, often going down one or both legs. 

7. Skin lesions: The condition can cause skin lesions if it affects the skin. However, mantle cell lymphoma skin involvement is extremely rare. 

Patients may also experience certain B-symptoms. They are called B-symptoms because they are clear symptoms of B-cell lymphoma. These symptoms include:

1. Fever and Night Sweats: Individuals with MCL may experience drenching night sweats and fever that do not go away with over-the-counter medications. It may happen due to the body’s immune response against lymphoma. 

2. Unexplained Weight Loss: MCL may cause unexplained weight loss despite normal eating habits. Individuals may lose at least 10% of their body weight within six months. This may result from increased energy expenditure and loss of appetite. 

The exact cause of mantle cell lymphoma (MCL) is not fully understood. However, mantle cell lymphoma pathophysiology involves certain gene mutations that change how the B-lymphocytes function. Mantle cell lymphoma translocation involves the exchange of small pieces of chromosomes 11 and 14. 

These mantle cell lymphoma mutations trigger the release of a protein called cyclin D, which plays a significant role in cell growth. The overload of this protein leads to the uncontrolled growth of B cells. These duplicate cells form tumours that lead to lymphomas. 

Mantle Cell Lymphoma Risk Factors 

Several risk factors influence the development of mantle cell lymphoma. However, one must note that having one or more risk factors does not guarantee the development of the disease. Here are some recognized risk factors associated with MCL:

1. Age: The risk of MCL is higher in older individuals, with most cases occurring in people above 60. According to an NCBI study of 51 MCL patients, the median age at diagnosis was 57 years. 45% of cases were reported in people over 60 years.

2. Gender: Men are about three times more likely than women to develop the disease. NCBI states that the male-to-female ratio of MCL among these patients was 2.4:1.

3. Viral Infections: Certain viral infections, like human immunodeficiency virus (HIV) or Epstein-Barr virus (EBV), have been associated with an increased risk of developing MCL. Few studies have found a connection between EBV and MCL. However, its role in MCL remains unknown.

4. Weakened Immune System: Those who have a weakened immune system due to genetic syndromes are at a higher risk of developing MCL. Individuals with weakened immune systems are less able to fight MCL-related infections.

Mantle cell lymphoma cannot be prevented because it occurs due to gene mutations. However, general healthy lifestyle choices may help reduce the risk of various types of cancer, including MCL. These lifestyle changes include:

1. Avoiding Exposure to Carcinogens: Individuals must reduce or avoid exposure to known carcinogens, like tobacco smoking, certain chemicals, or pesticides. 

2. Maintain a Healthy Lifestyle: One must exercise regularly, eat a balanced diet, manage stress, and get enough sleep to maintain a strong immune system. A healthy immune system helps prevent and fight cancer. 

3. Practise Safe Behaviours: One must protect oneself from viral infections like HIV by practising safe sex and taking other precautions. 

4. Get Regular Medical Checkups: Regular screenings and checkups can help detect the signs of MCL at an early stage. Individuals should follow the recommended cancer screening guidelines to protect themselves from the disease.

Healthcare providers begin the diagnosis by conducting a physical exam and evaluating symptoms. They may order the following tests and procedures to diagnose the disease following the mantle cell lymphoma pathology outlines: 

1. Blood Tests: These tests are conducted to evaluate the overall health of the individual and assess for any abnormalities. They can help measure the number of blood cells an individual has, the working of their liver and kidneys, and the presence of certain proteins in the blood that suggest MCL. These tests include:
   

1. Complete Blood Count (CBC): This blood test reveals the level of red blood cells (RBC), white blood cells (WBC), and platelets in an individual’s blood. It also measures the amount of haemoglobin in the rbc. 

2. Comprehensive Metabolic Panel (CMP): The test evaluates liver and kidney function. It also helps examine the blood electrolytes responsible for controlling nerve and muscle function. 

3. Lactate Dehydrogenase (LDH) Test: A blood sample is collected to check for tissue damage from a disease or injury. 

4. Uric acid level test: It measures the waste products in a person’s bloodstream.
   

2. Biopsy: The test involves removing a small sample of tissue from the tumour to be tested in a laboratory. Biopsies performed for mantle cell lymphoma include:

   1. Lymph Node Biopsy: A definitive diagnosis of MCL is typically made through a lymph node biopsy. The surgeon removes a small sample of the affected lymph node. The sample is then examined under a microscope to look for cancer cells. 
      
      Special tests, such as immunohistochemistry, may be performed on the biopsy sample to confirm the presence of specific mantle cell lymphoma markers. Common markers of MCL include CD19, CD20, CD22, CD79a, and BSAP/PAX5. 

   2. Bone Marrow Biopsy and Aspiration: Sometimes, a bone marrow biopsy may be performed to assess if cancer has spread to the bone marrow. This involves the extraction of a small sample of bone marrow from the hipbone, which is then examined for the presence of lymphoma cells.

3. Imaging Tests: Imaging tests are used to visualize the lymph nodes, organs, and other tissues to identify abnormalities. Mantle cell lymphoma radiology or imaging tests include:

   1. Computed Tomography (CT scan): The test uses X-rays to create 3D images of an individual’s soft tissues and bones. 

   2. Positron Emission Tomography (PET) scan: The test uses a radioactive substance to produce images of how organs and tissues are working. 

   3. Colonoscopy: In this test, the doctor inserts a thin, lighted tube in the patient’s rectum. The test helps detect signs of MCL in the colon and rectum. These signs may include polypoid lesions (abnormal tissue growths that protrude from the mucous membrane), bleeding, or obstruction. 

   4. Esophagogastroduodenoscopy (EGD): The test examines the signs of MCL in the upper gastrointestinal tract for patients with gastrointestinal issues. 

A lymph node biopsy can help diagnose MCL if additional examination of the tissue shows that the lymphoma cells have the following:

1. Mantle cell lymphoma CD markers of B-cells (e.g., CD 20)
2. A buildup of cyclin D1 protein within the cells
3. Changes in chromosomes 11 and 14

How to Prepare for Doctor’s Consultation?

Preparing for a consultation appointment about mantle cell lymphoma can help ensure a productive discussion with the healthcare provider. The following are some steps that can help individuals prepare:

1. Make an Appointment: Individuals should make a prior appointment. Also, they must be aware of any pre-appointment restrictions, such as limiting their diet. 

2. Enlist Symptoms and Medical History: It is advised to list the symptoms a person is experiencing. Healthcare providers may also ask questions about medical history. Therefore, patients must have information about the same. 

3. Make a List of Medications: Prepare a list of current medications, vitamins, and supplements, including dosages and frequencies. This information will be essential for the healthcare provider to assess potential interactions or contraindications with any prescribed treatments.

4. Prepare Questions: Jot down a list of questions and concerns to be discussed during the appointment. Consider questions related to treatment options, potential side effects, prognosis, and other queries. 

5. Take a Friend or Family Member Along: Patients should bring a trusted person to the appointment. They can provide support and help remember important information. 

What to Expect from the Doctor?

During a consultation for mantle cell lymphoma, the healthcare provider will likely ask the patient several questions. These questions can help them gather relevant information about their medical history, symptoms, and overall health. While the specific questions may vary, here are some questions one can expect:

1. What symptoms are you experiencing?

2. When did these symptoms start?

3. Are your symptoms continuous or occasional?

4. Does anything seem to improve or worsen these symptoms?

5. Have you noticed any changes in appetite or difficulty eating?

6. Do you feel unusually tired?

7. Have you lost weight recently?

8. Have you been diagnosed with any other medical conditions or had any previous cancers?

9. Are you currently taking any medications or have any known allergies?

10. Do you smoke or have a history of tobacco use?

Questions to Ask the Doctor

Patients must ask questions during the consultation appointment for MCL. This helps them gather relevant information, clarify doubts, and actively participate in treatment decisions. The following are some questions they may consider asking their doctor:

1. What is the cause of my symptoms?

2. What tests do I need for diagnosis?

3. What is the stage of my MCL? Is it localised, or has it spread to other organs?

4. Will I need treatment?

5. What are the recommended treatment options for my specific case of MCL?

6. Are there any alternatives to the treatments you are suggesting?

7. Are there any clinical trials available that I may consider?

8. How long will the treatment course typically last?

9. What are the potential side effects of the recommended treatments?

10. How can I manage other health conditions with mantle cell lymphoma?

11. Are there any specific dietary recommendations or restrictions I should follow?

12. Would it be beneficial to seek a second opinion from another specialist? How much will it cost?

Patients usually wonder, “Is there a cure for mantle cell lymphoma?” While a cure is not always possible, multiple treatment options are available to put the disease into remission. The treatment of mantle cell lymphoma depends on several factors, including the stage of the disease, the patient’s overall health, and individual preferences. 

1. Watchful Waiting: Doctors may suggest watchful waiting before starting treatment if an individual has a slow-growing form of cancer. However, most mantle cell lymphomas are aggressive and require immediate treatment. 

2. Chemotherapy: It is a standard treatment for MCL and involves using powerful drugs to kill cancer cells. Different chemotherapy regimens may be used, including combination drugs like cyclophosphamide, doxorubicin, vincristine, and prednisone. 

3. Targeted Therapy: This is designed to specifically target certain proteins involved in the survival and spread of cancer cells. The BCL2 inhibitor venetoclax for mantle cell lymphoma can be useful. It is particularly used in high-risk relapsed/refractory MCL. Monoclonal antibodies, such as rituximab, are another targeted therapy that uses lab-created antibodies to kill specific cancer cells. 

4. Immunotherapy: Healthcare providers may use immunotherapy drugs to enhance the body’s immune response against cancer cells. CAR T-cell therapy is an example of immunotherapy that helps treat cancer cells by turning T-cells into more efficient cancer-fighting machines. 

5. Autologous Stem Cell Transplant: For eligible patients with MCL, high-dose chemotherapy followed by stem cell transplantation may be considered. This procedure aims to replace the patient’s diseased bone marrow with healthy stem cells from their own body to restore normal blood cell production. However, it is not a mantle cell lymphoma cure. 

6. Clinical Trials: Medical researchers are investigating new treatments to increase remission time. Patients must consider participating in clinical trials to explore novel treatments or combinations of therapies to improve outcomes.

Please Note: The selection of the treatment approach is based on the patient’s condition and the treating doctor’s opinion. 

Cost of Mantle Cell Lymphoma Treatment

The cost of mantle cell lymphoma treatment in India is determined by several factors including the type and stage of the cancer, the location and type of the hospital, the doctor’s experience, the patient’s age, insurance coverage, and much more.

To know the exact cost of mantle cell lymphoma treatment in India, one needs to consult their doctor. The table below also provides the approximate cost of the treatment modalities used to treat mantle cell lymphoma

Mantle Cell Lymphoma Survival Rate

Mantle cell lymphoma survival rates depend on whether the condition is slow-growing or aggressive. The overall survival rates of MCL have doubled over the last decades. However, relapses are still common. 

1. People with MCL generally live for five to seven years after diagnosis. 

2. The average progression-free time is 20 months. According to an NCBI study of 51 MCL patients, the median follow-up time was 20.7 months. 

It is crucial to remember that survival rates are general estimates and do not predict the outcome for any individual case. The response to treatment and long-term prognosis can vary widely among individuals. The longest survivors of mantle cell lymphoma have even been alive for more than 20 years after diagnosis. 

Mantle Cell Lymphoma Prognosis

Generally, mantle cell lymphoma is considered an aggressive and challenging type of lymphoma with a less favorable prognosis than other types of non-Hodgkin lymphoma. However, advancements in treatment options have improved outcomes for many individuals with MCL.

It is important to note that each person’s prognosis is unique and can vary based on several factors, such as:

1. Mantle cell lymphoma international prognostic index (MIPI) rank

2. The stage of cancer

3. Treatment response

4. Patient’s overall health

Mantle cell lymphoma can lead to several complications. These complications vary depending on the stage of the disease, individual characteristics (age, overall health, etc.), and the specific organs affected. The following are some potential complications associated with MCL:

1. Splenic Rupture: It is a rare complication where the buildup of lymphoma cells in the spleen can lead to splenomegaly (spleen enlargement). This increases the chances of a ruptured spleen. 

2. GI Bleeding, Obstruction, or Perforation: Involvement of the gastrointestinal (GI) tract in MCL can cause complications like GI bleeding, obstruction, or perforation. MCL in the GI tract can cause polyps, stomach issues, or abdominal pain. 

3. Tumour Lysis Syndrome: This serious complication occurs when tumour cells release their content into the bloodstream. It usually develops in blastoid mantle cell lymphoma. 

4. Problems with Blood Cell Production: MCL can decrease healthy blood cells when the disease progresses. It can lead to anaemia (low red blood cell count), thrombocytopenia (low platelet count), and leukopenia (low white blood cell count). 

When to See a Doctor? 

Individuals must consult a healthcare professional if they experience any concerning symptoms or if they have specific risk factors associated with mantle cell lymphoma. The following are some symptoms that indicate a need to see a doctor:

1. Enlarged lymph nodes

2. Unexplained weight loss

3. Fever and night sweats

4. Persistent fatigue

5. Shortness of breath

6. Gastrointestinal problems

Furthermore, if patients are receiving treatment, they will need to visit their doctors regularly. The doctor will help manage treatment side effects, evaluate the effectiveness of treatment, and monitor the patient’s overall health.

No specific diet can treat or cure mantle cell lymphoma. However, a healthy and balanced diet is important to support overall health and well-being during and after treatment. The general dietary recommendations for individuals with MCL are similar to any healthy eating plan.

Foods to Eat

1. Plenty of Fluids: Drink an adequate amount of fluids, such as water, herbal teas, and clear broths, to maintain hydration. This is important for overall health and to manage potential side effects of treatments like chemotherapy.

2. Fruits and Vegetables: Individuals must include several fruits and vegetables in their diet to strengthen their immune systems. 

1. Examples of fruits include berries, melons, oranges, etc. 

2. Examples of vegetables include broccoli, cabbage, kale, etc. 

3. High Protein Food: Opt for protein sources like poultry, fish, lean red meat, eggs, nuts and beans. These foods can help promote healing and maintain muscle mass during treatment. 

4. Healthy Fats: It is advised to include food rich in healthy fats, like oily fish, avocados, and nuts, in one’s diet. They help provide energy, absorb essential nutrients, and support overall health.  

5. Carbohydrates: Carbohydrates provide energy, which is essential for MCL patients. Good sources of carbohydrates include oatmeal, whole-grain bread, and brown rice. 

If patients find it hard to eat during cancer treatment and experience weight loss, they may try to eat about five or six smaller meals throughout the day. It is better to eat foods that are rich in nutrients and calories, such as:

1. Granola bars

2. Soups, milkshakes, or nutritional drinks

3. Nuts and peanut butter

4. Eggs

5. Avocado

6. Butter and oil

7. Sauces and gravies

Foods to Avoid

Mantle cell lymphoma can reduce the level of white blood cells that help the body fight infections. Therefore, it is important to avoid foods that could contain bacteria, such as:

1. Uncooked or undercooked eggs, meat, and seafood

2. Unpasteurised milk and other dairy products

3. Yoghurt with active cultures (a gut-friendly bacteria used to convert milk to yoghurt)

4. Soft cheese, like blue cheese

Mantle cell lymphoma,a rare, aggressive type of non-Hodgkin lymphoma, typically requires comprehensive and individualised treatment approaches.

Prognosis and survival rates vary depending on various factors, including stage, age, and treatment response. Advances in treatment options, such as targeted therapies and stem cell transplantation, have improved outcomes for some individuals. 

Are you looking for expert guidance and support to manage mantle cell lymphoma? Look no further! The team at HexaHealth offers personalised treatment plans and ongoing monitoring to ensure comprehensive care.

Our experts offer accurate diagnosis utilising resources like “mantle cell lymphoma pathology outlines” to help with prompt treatment and management of the disease. Contact us TODAY to know more! 

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