Mantle cell lymphoma (MCL) is a rare type of blood cancer that affects the lymphocytes (white blood cells) that protect your body from infection. 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 what your prognosis (outlook) is 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. These cells make antibodies, or proteins, that tag invading bacteria and viruses to be destroyed by your immune system. B cells are found in lymph nodes around your body, typically in a specialized region known as the mantle zone.

Mutations or genetic changes in B cells cause them to grow and divide uncontrollably in the mantle zone, leading to MCL. Compared with other types of NHL, MCL is a fairly aggressive type of lymphoma. At first, the cancer cells grow slowly and are limited to the lymph nodes. Over time, they begin growing and dividing rapidly, spreading to other parts of the body such as the bloodstream, liver, bone marrow, and gastrointestinal tract.

At Which 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 doctors aren’t aware of the signs, so it’s often misdiagnosed as another, milder condition.

The longer it takes to receive a diagnosis, the more time the cancer has to continue growing. As a result, around 70 percent of people with MCL are diagnosed at stage 4. This means that their cancer has spread outside of the lymph nodes into other parts of their body.

Mantle Cell Lymphoma Symptoms To Know

The most common MCL symptom is having painless swollen lymph nodes, which occurs 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 — instead, 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 cause a particular set of symptoms called 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 doctor.

It’s not uncommon for people to receive an MCL diagnosis following routine blood work. A complete blood count panel is a standard blood test, and a high white blood cell count could point to MCL. The best way to monitor your health is to go to your annual checkups and get blood work done as recommended by your doctor.

Treatments for Late-Stage Mantle Cell Lymphoma

Treatment options differ depending on the stage of MCL at diagnosis. An early-stage diagnosis is rare, but stages 1 and 2 MCL may be treated with chemotherapy, rituximab (Rituxan), and/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 (Cytoxan), doxorubicin (Adriamycin), vincristine (Oncovin), and prednisone, alternated with the chemotherapy drug cytarabine and rituximab
• Hyper-CVAD — Cyclophosphamide, vincristine, doxorubicin, and dexamethasone, alternated with high-dose methotrexate plus cytarabine
• R-DHAP — Rituximab, dexamethasone, cytarabine, and cisplatin

A combination of rituximab and bendamustine (Treanda) may be used to treat those 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 (Velcade) and ibrutinib (Imbruvica).

Treatments for Relapsed or Refractory Mantle Cell Lymphoma

Stage 4 MCL is often difficult to treat, and it may relapse (return) after you complete your chemotherapy regimen. It can also become resistant or refractory to chemotherapy, meaning you don’t go into remission (a period of having fewer or no symptoms). Your oncologist may recommend:

• Another targeted therapy, such as venetoclax (Venclexta), zanubrutinib (Brukinsa), or acalabrutinib (Calquence)
• Chimeric antigen receptor T-cell therapy (CAR T-cell therapy), such as brexucabtagene autoleucel (Tecartus)
• Other chemotherapies, such as rituximab plus lenalidomide (Revlimid)

Prognosis With Late-Stage Mantle Cell Lymphoma

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

Your prognosis can be examined in terms of overall survival, which refers to the number of people with a disease who are alive after a set amount of time. Studies show that the five-year overall survival rate for advanced-stage MCL is around 50 percent. This means that after five years, 50 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 14,000 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.