Mantle cell lymphoma (MCL) is a form of cancer that affects the white blood cells, or lymphocytes, that protect your body from infection. This rare type of blood cancer is diagnosed in about 1 in 200,000 people each year and makes up about 5 percent of all non-Hodgkin lymphomas. MCL can be an aggressive form of cancer that tends to be diagnosed at later stages. Most people don’t have symptoms until the disease has progressed, making MCL difficult to diagnose early.

Read on to learn more about the reasons MCL is typically diagnosed at later stages, signs to look out for, and your treatment options. We’ll also discuss your prognosis (outlook) after an advanced-stage MCL diagnosis.

What Is Mantle Cell Lymphoma?

MCL is a rare type of non-Hodgkin lymphoma (NHL) that develops from specialized white blood cells known as B cells. When activated, B cells produce proteins called antibodies that tag invading bacteria and viruses to be destroyed by your immune system. B cells are found in lymph nodes throughout your body, typically in a specialized region known as the mantle zone. In MCL, genetic changes cause these B cells to grow and divide out of control. One common change is a switch in genes, called a translocation, that affects how B cells grow. This uncontrolled growth in the mantle zone leads to MCL.

Is Mantle Cell Lymphoma an Aggressive Cancer?

Compared with other subtypes of NHL, MCL is a fairly aggressive type of lymphoma. At first, the cancer cells grow slowly within the lymph nodes. However, they can eventually multiply rapidly and spread to other parts of the body, including the liver, bone marrow, and gastrointestinal tract. MCL can be categorized into subtypes based on how the cancer cells look under a microscope. The blastoid variant, which is the most aggressive form of these MCL subtypes, is linked with poor outcomes.

At What Stage Is Mantle Cell Lymphoma Usually Diagnosed?

MCL is difficult to diagnose at an early stage because many people don’t experience symptoms until the cancer has progressed and spread. MCL is also a rare type of cancer, with around 4,000 new cases diagnosed in the U.S. every year. Many healthcare providers may not recognize the signs, leading to MCL being misdiagnosed as another, milder condition.

The longer it takes to be diagnosed, the more time the cancer has to keep growing. As a result, around 70 percent of people with MCL are diagnosed at stage 4 (also written as stage IV). This means the cancer has spread outside the lymph nodes into other parts of the body.

What Are the First Signs of Mantle Cell Lymphoma?

The most common MCL symptom involves painless swollen lymph nodes, which occur in around 90 percent of people with the disease. Lymph nodes in the armpits, groin, and neck are typically affected. You’re less likely to notice bumps under the skin of your armpits and groin, but you might see or feel them in your neck. A bacterial or viral infection can also cause swollen lymph nodes in the neck, so it’s easy to think your body is just fighting a routine illness.

Different types of NHL are linked with a specific set of symptoms known as B symptoms, which include:

• Unexplained fever
• Sudden weight loss
• Drenching night sweats

Although B symptoms are often a telltale sign of MCL, only 40 percent of people with the condition will experience them. If you’ve noticed any of these symptoms for at least two weeks, be sure to mention them to your oncology (cancer) care team.

MCL is sometimes identified through routine blood tests, such as a complete blood count (CBC). In MCL, the CBC may reveal high white blood cell counts, anemia (low red blood cell levels, which can cause pale skin), or thrombocytopenia (low platelet levels, which can cause easy bruising). Attending regular checkups and following your doctor’s recommendations for blood work are important ways to catch potential health issues early.

How Is Late-Stage Mantle Cell Lymphoma Treated?

Treatment options differ depending on your MCL stage at diagnosis, your overall health, and other factors. An early-stage diagnosis is rare, but stages 1 and 2 MCL may be treated with chemotherapy, rituximab (Riabni, Rituxan, Ruxience, Truxima), or radiation therapy.

Since MCL is typically diagnosed at stage 4, significant research has gone into treating advanced-stage disease. Treatment usually starts with chemoimmunotherapy, a powerful combination of chemotherapy drugs plus immunotherapy to stop cancer cells from multiplying. Examples of chemoimmunotherapy used to treat MCL include:

• Dose-intensified R-CHOP — Rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (Prednisone Intensol, Rayos), alternated with high-dose cytarabine (a chemotherapy drug) and rituximab
• Hyper-CVAD — Cyclophosphamide, vincristine, doxorubicin, and dexamethasone (Dexabliss), alternated with high-dose methotrexate (Jylamvo, Otrexup, Rasuvo) and cytarabine
• R-DHAP — Rituximab, dexamethasone, high-dose cytarabine, and cisplatin

A combination of rituximab and bendamustine (Bendeka, Treanda) may be used to treat people who can’t tolerate intense chemotherapy treatments, including older individuals with MCL or those who have other health conditions.

Targeted therapies that specifically block proteins involved in MCL include bortezomib (Boruzu, Velcade) and lenalidomide (Revlimid).

What Are the Treatments for Relapsed or Refractory Mantle Cell Lymphoma?

Stage 4 MCL is often difficult to treat, and it may relapse (return) after the completion of your chemotherapy regimen. It can also become refractory (resistant) to chemotherapy, meaning it doesn’t respond to treatment or go into remission (a period with fewer or no symptoms). Your oncologist (cancer doctor) may recommend:

• Another targeted therapy, such as acalabrutinib (Calquence), pirtobrutinib (Jaypirca), venetoclax (Venclexta) — which is used off-label — or zanubrutinib (Brukinsa)
• Chimeric antigen receptor (CAR) T-cell therapy, such as brexucabtagene autoleucel (Tecartus) or lisocabtagene maraleucel (Breyanzi)
• Chemotherapies that you haven’t used previously
• Immunotherapy drugs, such as lenalidomide plus rituximab

What Is the Prognosis With Late-Stage Mantle Cell Lymphoma?

Receiving a late-stage cancer diagnosis can be scary and overwhelming. Your prognosis with MCL can be affected by several factors, including disease stage, your age, and specific mutations (changes) found in your lymphoma cells.

How Serious Is Mantle Cell Lymphoma?

Prognosis is often described in terms of overall survival, which refers to the percentage of people with a disease who are alive after a specific amount of time. Studies show that the five-year overall survival rate for advanced-stage MCL is around 65 percent. This means that after five years, 65 percent of people with MCL are alive.

New treatment advances are helping people with MCL live longer, healthier lives, and the prognosis continues to improve over time. If you’re interested in learning more about your outlook with MCL, talk with your oncologist.

Talk With Others Who Understand

On MyLymphomaTeam, the social network for people with lymphoma and their loved ones, more than 21,000 members come together to ask questions, give advice, and share their stories with others who understand life with lymphoma.

Are you or a loved one living with mantle cell lymphoma? At which stage did you get the diagnosis? Share your questions or experiences in the comments below, or start a conversation by posting on your Activities page.