Mantle Cell Lymphoma

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At Dana-Farber’s Mantle Cell Lymphoma Center, our team of specialists is focused on delivering advanced, personalized care to patients with mantle cell lymphoma. We are one of a select few centers nationwide dedicated to advancing research efforts to better understand and treat this cancer.  

Why Choose Dana-Farber's Mantle Cell Lymphoma Center

Our team at the Mantle Cell Lymphoma Center has extensive experience and expertise, combining lab and clinical research to better understand this specific lymphoma type and optimize care for patients.

Meet Our Experts

We focus on delivering compassionate, patient-centered care that addresses the specific challenges faced by those with mantle cell lymphoma. Services include: 

  • Comprehensive evaluation
  • Expert second opinions
  • Advanced diagnostics (pathologic)
  • Individualized treatment plans offering the latest therapies
  • Individualized treatment plans offering the latest therapies, including targeted combination therapies and immunotherapies like CAR T-cell therapy  
  • Clinical trials offering novel therapies, some only available at Dana-Farber
  • Comprehensive supportive care including psychosocial and palliative services
  • Access to complementary therapies like nutrition counseling, acupuncture, and massage
  • Dedicated follow-up care, including collaboration with your local (referring) physician 

What Is Mantle Cell Lymphoma?  

Mantle cell lymphoma is a form of non-Hodgkin B-cell lymphoma. It develops in the mantle cell, a ring of cells within the lymph nodes where B cells—a type of lymphocyte—grow. Most patients with mantle cell lymphoma have an aggressive form of the disease that can spread throughout the bloodstream, digestive system, and bone marrow. Approximately 10-20 percent of patients with mantle cell lymphoma have a slow-growing, non-aggressive version of the disease.  

A key aspect of mantle cell lymphoma is the variation observed in patients with this disease. How the lymphoma affects a given patient, and how a patient responds to treatment is highly variable between different individuals. This highlights the need for deeper insights to inform new treatment approaches that are more broadly effective or that can be matched to patients who are most likely to benefit.   

What Are Causes and Risk Factors of Mantle Cell Lymphoma?

Mantle cell lymphoma occurs when B lymphocytes acquire genetic mutations that alter their function and growth. The most common abnormality, found in 90 percent of cases, causes B lymphocytes to overproduce cyclin D1, a protein that drives the cells’ growth. Other mutations can interfere with B cells’ ability to overcome DNA damage and produce infection-fighting antibodies, leaving patients vulnerable to treatment resistance and long-term complications such as low blood counts and infections.

Mantle cell lymphoma is usually first diagnosed in individuals between the ages of 60 to 70 years. Its cause is unknown for most patients, but farmers and people from rural areas are more likely to develop mantle cell lymphoma, suggesting that environmental exposures may play a role in some patients. 

What Are Mantle Cell Lymphoma Symptoms and Signs?

The most common symptoms of mantle cell lymphoma are:

  • Night sweats
  • Fever
  • Unexplained weight loss
  • Enlarged lymph nodes in the neck, groin, or armpits
  • Symptoms related to an enlarged spleen, tonsils, or liver, such as feeling full more quickly than usual when eating a meal

Patients with mantle cell lymphoma can also experience a variety of other symptoms often mistaken as common physical problems, including:

  • Nausea
  • Vomiting
  • Diarrhea
  • Fatigue

How Is Mantle Cell Lymphoma Diagnosed?

Tests used to diagnose and evaluate mantle cell lymphoma can include:  

  • Blood tests, including complete blood count, comprehensive metabolic panel, and lactate dehydrogenase test (LDH)
  • Imaging tests, such as PET/CT scans to identify where lymphoma is present in the body
  • Lymph node and bone marrow biopsy  
  • Colonoscopy or esophagogastroduodenoscopy to examine signs of mantle cell lymphoma in the body’s gastrointestinal system

At the time of diagnosis, nearly all patients have disease that has spread beyond one lymph node group in the body.

What Are the Stages of Mantle Cell Lymphoma?

Lymphoma staging describes how much the cancer has spread in your body and helps guide treatment decisions. It uses a scale from I to IV, with higher numbers indicating more widespread disease.  

  • Stage I means cancer is found in only one lymph node area or organ.
  • Stage II indicates cancer in two or more lymph node groups, but only on one side of the diaphragm.
  • Stage III means the cancer has spread to lymph node regions or structures on both sides of the diaphragm.
  • Stage IV indicates that the lymphoma involves organs outside the lymph system including the bone marrow. 
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The stages of Lymphoma as it progresses in the body, from Stage I to Stage IV.

How Is Mantle Cell Lymphoma Treated?

Treatment selection for mantle cell lymphoma is based on a patient’s characteristics such as age, medical conditions, and symptoms, as well as disease features such as stage, proliferation rate, and genetic mutations. Choosing a treatment plan also depends on whether the patient is newly diagnosed, or has disease that has grown back after prior therapy. Our team takes a personalized approach to care, identifying the most effective and tolerable treatment options for each patient.

Active Surveillance: How We Monitor and Manage Disease Progression 

Some people diagnosed with mantle cell lymphoma may not need immediate treatment if they do not have symptoms. These patients may be recommended for “active surveillance” — close monitoring of their health through regular checkups and lab tests. When lymphoma-related symptoms appear or tests show a worsening of the disease, active treatment may begin.  

Patients who follow an active surveillance treatment plan can expect to meet with their doctor every 3-4 months to monitor for disease progression or development of symptoms. Visits may also be more frequent if needed.

Treatment for Newly Diagnosed Mantle Cell Lymphoma

For patients who require treatment, initial treatment in younger patients includes upfront intensive chemotherapy in combination with an antibody-based treatment, followed by maintenance treatment with antibodies and/or oral targeted therapies. Older patients may receive less intensive chemotherapy in combination with an antibody-based treatment often followed by maintenance treatment. Chemotherapy-free treatment approaches are also being used in certain situations.

Our center is involved in research and clinical trials to improve the initial treatment for mantle cell lymphoma. This includes evaluation of drugs known as BTK inhibitors which interfere with lymphoma cells’ internal growth signals. Our overall goal is to establish treatment approaches that maximize the duration of time a patient’s lymphoma remains in remission after initial treatment while minimizing treatment side effects.

Treatment for Relapsed/Refractory Mantle Cell Lymphoma

Patients with aggressive, relapsed, or refractory mantle cell lymphoma may be treated with:  

  • Targeted drug therapy, such as acalabrutinib, zanubrutinib, pirtobrutinib, venetoclax, and lenalidomide
  • CAR T-cell therapy
  • Bispecific antibody therapy
  • Monoclonal antibody therapy in combination with chemotherapy
  • Novel therapies as part of a clinical trial

Our center is focused on developing better treatments for those who have relapsed/refractory mantle cell lymphoma, including for those whose disease has high-risk genetic mutations. While initial treatment for the disease is often successful, most patients eventually relapse, highlighting the need for better treatments. Our team is dedicated to improving treatments and outcomes for patients with mantle cell lymphoma. We have trials for patients with relapsed/refractory mantle cell lymphoma exploring new treatments such as targeted combination therapies and novel immune-based therapies.