Diffuse Large B-Cell Lymphoma

What is diffuse large B-cell lymphoma?

Diffuse large B-cell lymphoma (DLBCL) is a fast-growing blood cancer and the most common form of non-Hodgkin lymphoma. There are different forms of DLBCL that happen when several different genetic changes, or mutations, turn healthy cells into cancerous cells.

Healthcare providers treat most DLBCL types by combining several cancer drugs. This treatment often eliminates DLBCL signs and symptoms and cures the condition. The drug combination doesn’t work for all cases of diffuse large B-cell lymphoma. Medical researchers are studying different treatments for DLBCL that don’t respond to treatment or comes back.

How does diffuse large B-cell lymphoma affect my body?

To understand how DLBCL may affect you, it may help to know more about non-Hodgkin lymphomas. These conditions happen when white blood cells known as lymphocytes mutate or change. These cells include B cells, T cells and natural killer (NK) cells.

There are two categories of non-Hodgkin lymphoma — B-cell lymphoma and T-cell/NK lymphoma. Diffuse large B-cell lymphoma is a B-cell lymphoma.

B-cell lymphomas frequently affect your lymphatic system. Your lymphatic system is a network of tissues, vessels and organs that work together to move a colorless, watery fluid called lymph back into your bloodstream. Lymph contains B cells (B lymphocytes). B cells make antibodies. Antibodies are proteins that target intruders such as viruses and bacteria.

Normally, healthy B cells die when they’re done fighting infection. B-cell lymphoma happens when healthy B cells change into fast-growing cancer cells that don’t die the way that they should. The cancer cells duplicate, eventually overwhelming healthy cells.

In diffuse large B-cell lymphoma, cancerous B cells may appear in your lymph nodes, but in some cases, they can appear in virtually any organ, including your gastrointestinal tract, thyroid, skin, breast, bone or brain. Diffuse large B-cell lymphoma is aggressive, meaning it may spread very quickly.

Is it a serious illness?

It can be, depending on your situation. One study showed a combination of cancer drugs used as front-line or initial treatment cured 90% of people with early or limited-stage lymphoma. (Early-stage cancer is cancer that hasn’t spread.) This same treatment helped up to 60% of people with a more advanced form of diffuse large B-cell lymphoma. However, the combination treatment doesn’t cure all DLBCL and it may not keep the condition from coming back (recurring).

Is diffuse large B-cell lymphoma common?

No, it’s not, at least compared to other forms of cancer. According to the National Cancer Institute, in 2020, about 6 people in 100,000 were diagnosed with DLBCL. In comparison, that year, 19 people in 100,000 were diagnosed with some form of non-Hodgkin lymphoma, and about 500 people in 100,000 were diagnosed with cancer affecting any part of their bodies.

Who’s affected by this condition?

Diffuse large B-cell lymphoma typically affects people in their 60s. It affects more people who are white than people who are Black.

What are diffuse large B-cell lymphoma symptoms?

The symptoms most people notice are swollen lymph nodes in their neck, armpits or groin. They may notice a lump or mass that doesn’t go away and seems to be getting larger. Other symptoms sometimes include:

• Unexplained fever: A fever that stays above 103 degrees Fahrenheit (39.5 degrees Celsius) that lasts longer than two days or that comes back may be a sign of a serious issue.
• Heavy night sweats: This is sweating that’s so intense it drenches your sheets.
• Unexplained weight loss: This is losing body weight without trying. Losing 10% of your total body weight over six months is a sign of unexplained weight loss.

It’s important to remember these are common symptoms related to many different conditions. Having one or all of these symptoms doesn’t mean you have diffuse large B-cell lymphoma. That said, you should contact a healthcare provider anytime you notice changes in your body that last for several weeks.

What causes diffuse large B-cell lymphoma?

Diffuse large B-cell lymphoma happens when B cells mutate. These are acquired genetic mutations, meaning you develop them during your lifetime instead of being born with them.

Medical researchers aren’t sure what triggers these mutations, but having a family history of DLBCL could be a risk factor. A risk factor is an activity or condition that increases your risk of developing a disease. Other risk factors may include:

• Certain viruses, including Epstein-Barr virus, human immunodeficiency virus (HIV), hepatitis B and hepatitis C.
• Immunosuppressant drugs taken after organ transplants.
• Autoimmune disorders.
• Immunodeficiency.
• Increased body mass index in young adults.
• Agricultural pesticides.
• Ionizing radiation.

How do healthcare providers diagnose diffuse large B-cell lymphoma?

Healthcare providers use several different blood tests and an imaging test to diagnose this condition. They also do genetic testing to understand whether specific mutations, or changes, are part of how a given case of diffuse large B-cell lymphoma occurred.

Tests healthcare providers may use include:

• Detailed physical examination.
• Complete blood count (CBC) with differential.
• Comprehensive metabolic profile (CMP).
• Lactate dehydrogenase (LDH).
• Human immunodeficiency virus (HIV/AIDS).
• Hepatitis B and Hepatitis C virus.
• Lymph node biopsy.
• Positron emission tomography (PET) scan and/or computed tomography (CT) scan.

Why are genetic tests important when diagnosing DLBCL?

Diffuse large B-cell lymphoma happens when genes mutate, or change. In DLBCL, the mutations may affect several different genes and chromosomes to create different types of diffuse large B-cell lymphoma.

• Diffuse large B-cell lymphoma (DLBCL-NOS): NOS stands for “not otherwise specified.” There are two forms of DLBCL-NOS: germinal center B-cell-like (GCB) and activated B-cell-like (ABC). These forms have different genetic makeups that affect how they respond to treatment. GCB represents about 50% of DLBCL-NOS and is more likely to respond to treatment than ABC. Other subtypes include double-hit lymphoma and triple-hit lymphoma, named for the combined impact of genetic mutations and chromosomal changes that create cancer cells.
• Primary mediastinal B-cell lymphoma: People with this subtype have fast-growing lymphoma in the center of their chests. They may also have lymphoma growths in other parts of their bodies.
• CNS lymphoma: This diffuse large B-cell lymphoma affects people’s central nervous systems.

Stages of diffuse large B-cell lymphoma

Test results help providers with cancer staging. Healthcare providers use cancer staging systems to develop treatment plans and estimate prognosis, or expected outcome. Here’s information about DLBCL stages:

Stage I

• There’s lymphoma in one lymph node area or one lymphoid organ. Your thymus, spleen and tonsils are lymphoid organs.
• Stage IE: There’s lymphoma in just one area of a single organ outside of your lymph system.

Stage II

• There’s lymphoma in two or more groups of lymph nodes on the same side of (above or below) your diaphragm, which is the band of muscle separating your chest and belly.
• Or the lymphoma is part of a group of lymph nodes and is in one area of a nearby organ. In this stage, the lymphoma may also affect other lymph nodes near your diaphragm.

Stage III

• There’s lymphoma in lymph node areas on both sides of (above and below) your diaphragm.

Stage IV

• There’s lymphoma in at least one area outside of your lymph system, such as your bone marrow, liver or lung.

Understanding cancer staging

Healthcare providers use cancer staging systems to plan treatment for diffuse large B-cell lymphoma.

Some people who have cancer may be confused and intimidated by a system that describes their illness with a formula of letters and numbers or risk factors that place them in one category or another. They may even feel as if their condition’s stage or category defines who they are.

If this is your situation, talk to your provider. They’ll understand why you may feel this way and will be glad to answer your questions about cancer staging systems.

How do healthcare providers treat diffuse large B-cell lymphoma?

The standard treatment is R-CHOP. It combines the monoclonal antibody drug rituximab with three chemotherapy drugs and a drug that helps kill lymphoma cells.

This treatment is safe and effective but doesn’t always work or keep DLBCL from coming back. (Studies show relapses or recurring disease affect an estimated 10% to 35% of people who receive R-CHOP.) If that happens, healthcare providers may recommend the following treatments:

• Second-line therapy and autologous stem cell transplant: Second-line therapy is intensive treatment with combined cancer drugs.
• Chimeric antigen receptor (CAR) T-cell therapy: This treatment may be helpful for people with diffuse large B-cell lymphoma that comes back after treatment.
• Targeted therapy: This treatment targets the genetic changes or mutations that cause diffuse large B-cell lymphoma.

Can I reduce my risk of developing diffuse large B-cell lymphoma?

A risk factor is an activity or condition that increases your risk of developing a condition. There are DLBCL risk factors you can’t control, like having an autoimmune disease or immunodeficiency. And there are risk factors you may be able to avoid, like developing certain infections.

Avoiding risk factors may help, but there’s no guarantee you won’t develop DLBCL. That’s because this condition happens when certain genes mutate and create cancerous cells. These are acquired genetic mutations, meaning you develop them during your lifetime instead of being born with them. Medical researchers aren’t sure why these mutations happen or the specific role that risk factors may play.Additional risk factors for diffuse large B-cell lymphoma include:

• Immunosuppressant drugs taken after organ transplants.
• Exposure to agricultural pesticides.
• Exposure to ionizing radiation.

What can I expect if I have diffuse large B-cell lymphoma?

That depends on your situation. Like many kinds of cancer, healthcare providers have more success treating early-stage DLBCL than advanced or later-stage cancer that’s spread. In general, people who are cancer-free two years after their diagnosis can expect to live as long as most people in their age group.

However, diffuse large B-cell lymphoma can come back. And there are different forms of DLBCL that are more difficult to treat. If you have this condition, your healthcare provider is your best source of information about what you can expect.

Are there treatments that can cure this condition?

Many times, front-line or initial treatment cures people with early-stage diffuse large B-cell lymphoma. (In this case, “cure” means that treatment eliminated all signs and symptoms of cancer.) Front-line or initial treatment cures about 60% of people who have advanced or later-stage DLBCL.

What are survival rates for diffuse large B-cell lymphoma?

According to the National Cancer Institute, 64.6% of people with DLBCL are alive five years after diagnosis. (Relative survival rates are estimates based on large groups of people.)

Like many kinds of cancer, survival rates increase for people with early-stage cancer. In this case, 79.5% of people with Stage I DLBCL are alive five years after diagnosis. Often, people have advanced-stage cancer by the time they receive a diagnosis. In that case, 54.7% of people with Stage IV DLBCL are alive five years after diagnosis.

It’s important to remember survival rates are estimates. They’re based on the experiences of large groups of people who have with different forms of DLBCL, at different times in their lives and different overall health.

Survival rate estimates may be helpful, but your healthcare provider is your best resource if you have questions about what you may expect.

How do I take care of myself?

Like most forms of cancer, it can be stressful and exhausting to live with diffuse large B-cell lymphoma. It’s important that you do what you can to take care of yourself throughout your treatment for diffuse large B-cell lymphoma. Here are some suggestions:

• Eat a healthy diet.
• Get as much rest as you can.
• Develop an exercise routine. Gentle exercise may help you cope with stress.
• Find support. Diffuse large B-cell lymphoma is a rare condition. You may feel as if no one understands what you’re going through. Connecting with people who are in your situation may help.

When should I see my healthcare provider?

You’ll see your healthcare provider throughout your treatment. They’ll monitor your overall health. They’ll assess how you’re reacting to treatment and whether the treatment is working. You may see your provider every three to four months for the first two years and then less frequently for the next three years. That’s because diffuse large B-cell lymphoma may come back. Contact your healthcare provider if you notice changes in your body that may mean your condition has come back.

When should I go to the emergency room?

Cancer treatment may have strong side effects. Your healthcare provider may have given you medication to help manage your side effects. You should go to the emergency room if you have:

• Side effects that are stronger than you expected.
• A fever higher than 100.4 degrees Fahrenheit (38 degrees Celsius).
• Chills that don’t go away.
• Abdominal pain.
• Persistent diarrhea.

What questions should I ask a healthcare provider about diffuse large B-cell lymphoma?

Diffuse large B-cell lymphoma is a rare disease. If you have this condition, you may want to ask your healthcare provider some of the following questions:

• What type of diffuse large B-cell lymphoma do I have?
• What’s this cancer’s stage?
• What treatment do you recommend?
• What are treatment side effects?
• What can I expect from treatment?
• Will this treatment cure my condition?
• What are the chances this condition will come back after treatment?
• What treatments are available if my condition comes back?
• Are there clinical trials for different treatments?

A note from Cleveland Clinic

Diffuse large B-cell lymphoma (DLBCL) is a fast-growing blood cancer and the most common form of non-Hodgkin lymphoma. Most of the time, healthcare providers can cure this condition, using a combination of cancer drugs as initial or front-line therapy. (In this case, “cure” means that treatment eliminated cancer signs and symptoms.)

Unfortunately, there are situations when initial therapy doesn’t work. Diffuse large B-cell lymphoma can come back after treatment. When that happens, healthcare providers have different treatments that may be successful.

At the same time, medical researchers are testing many treatments that may do more to help people with DLBCL. If you have this condition and want information about newer treatments, ask your healthcare provider about participating in a clinical trial.