Diffuse Large B-Cell Lymphoma

What is diffuse large B-cell lymphoma?

Diffuse large B-cell lymphoma, or DLBCL, is a blood cancer that involves changes in your B cells, a particular type of white blood cell (lymphocyte). It’s the most common form of aggressive non-Hodgkin lymphoma and a type of B-cell lymphoma.

DLBCL affects your lymphatic system. Your lymphatic system is a network of tissues, vessels and organs that help fight infection in your body. Normal B cells are a part of that infection-fighting network. But with DLBCL, healthy B cells change into fast-growing cancer cells that overtake healthy ones. They’re no longer able to fight off infection-causing invaders, like viruses and bacteria.

With diffuse large B-cell lymphoma, cancerous B cells may appear in your lymph nodes. But they may also appear in virtually any organ, including your gastrointestinal tract, thyroid, skin, breast, bone or brain.

Although it’s aggressive, DLBCL is often treatable and curable — especially with early diagnosis and treatment.

Types of DLBCL

The World Health Organization (WHO) has identified over a dozen different types of DLBCL. Each type’s unique traits tell healthcare providers how the cancer will likely progress and respond to treatment. The classifications depend on things like:

• Genetic changes. The genetic changes in the lymphoma cells are the most important identifier.
• The part of your body where DLBCL starts. For example, primary CNS lymphoma starts in your central nervous system (CNS). Primary mediastinal B-cell lymphoma starts in the center of your chest (mediastinum).
• If there’s an association with a virus. Some types of DLBCL happen in people with a specific virus. For example, people with EBV-positive DLBCL have an Epstein-Barr virus infection.

Understanding how the specific type of diffuse large B-cell lymphoma will impact your care journey is important, but the specifics can be confusing. Ask your provider to explain how your type of DLBCL will impact treatment options and outlook.

How common is diffuse large B-cell lymphoma?

Diffuse large B-cell lymphoma is the most common type of lymphoma. But as cancer diagnoses go, it’s still uncommon overall. According to the National Cancer Institute, in 2020, about 6 people in 100,000 received a DLBCL diagnosis. In comparison, about 500 people in 100,000 received a diagnosis of cancer affecting any part of their bodies.

What are the symptoms of DLBCL?

The symptoms most people notice with diffuse large B-cell lymphoma are swollen lymph nodes in their neck, armpits or groin. They usually appear as a lump that doesn’t go away and seems to be getting larger. The lump isn’t usually painful, but it can be.

About 30%of people with DLBCL have “B symptoms,” which include:

• A fever above 103 degrees Fahrenheit (39.5 degrees Celsius) that lasts longer than two days or comes and goes
• Unexplained weight loss that involves losing more than 10% of your body weight over six months
• Heavy night sweats (so intense that it drenches your sheets)

Having these symptoms doesn’t necessarily 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 (change). These are acquired genetic mutations, meaning you develop them during your lifetime instead of being born with them.

Risk factors

Medical researchers aren’t sure what triggers the mutations in DLBCL, but they’ve identified several factors that may increase your risk. Risk factors for diffuse large B-cell lymphoma include:

• Age. Most people diagnosed with DLBCL are in their 60s. The average age of diagnosis is 64 years old.
• Sex. DLBCL is slightly more common in people assigned male at birth (AMAB).
• Race. DLBCL affects more people who are white than people who are Black.
• Family history of DLBCL. The genetic mutations in DLBCL aren’t hereditary, which means, they don’t run in families. Still, studies show you’re at a slightly higher risk if a first-degree biological relative (parent, sibling or child) also has DLBCL. Medical researchers aren’t sure why.
• Infections. Viral infections associated with DLBCL include Epstein-Barr virus, human immunodeficiency virus (HIV) and hepatitis B and C.
• Weakened immune system. Having a condition that impacts your immune system is the most significant risk factor for DLBCL. Examples include having a primary immunodeficiency or autoimmune disorder, or taking immunosuppressants after an organ transplant.
• Increased body mass index (BMI) in young adults. The risk is especially high for young adults with obesity that persists throughout adulthood.
• Exposure to toxic substances. Exposure to pesticides used in farming and certain chemicals used in manufacturing may increase your risk.

How is diffuse large B-cell lymphoma diagnosed?

Healthcare providers diagnose DLBCL with a lymph node biopsy. This procedure removes all or part of a lymph node to check it for cancer cells. They also do genetic testing to learn which cell mutations are involved.

If you do have DLBCL, your provider may order additional tests to learn more about it, including whether it’s spread beyond your primary lymph node. Tests include:

• Blood tests. These include a complete blood count (CBC) to check your general health and tests that detect viruses, like HIV, EBV and hepatitis B and C.
• Lactate dehydrogenase (LDH) test. This test checks the level of LDH (an enzyme) in your body fluid. Over 50% of people with DLBCL have high levels.
• Imaging tests. You may need a CT scan, MRI or PET scan to show whether the cancer has spread throughout your body.
• Bone marrow biopsy. This test checks for lymphoma cells in your bone marrow.
• Lumbar puncture. This test checks for lymphoma cells in the fluid surrounding your spinal cord.

Stages of diffuse large B-cell lymphoma

Healthcare providers use cancer staging systems to determine how advanced the cancer is. This helps them develop treatment plans and estimate prognosis, or expected outcome. The stages of DLBCL are:

• Stage I: The cancer is in one lymph node, one lymph organ (thymus, spleen and tonsils) or in just one area of a single organ outside of your lymph system.
• Stage II: This is when there’s lymphoma in two or more lymph node groupings or lymph node organs on the same side of your diaphragm.
• Stage III: This stage is when there’s cancer in lymph nodes or lymph tissue on both sides of your diaphragm.
• Stage IV: DLBCL has spread to organs outside of your lymph system, like your bone marrow, liver or lungs.

Your provider may refer to stage I and II DLBCL as “early stage” and stage III and IV DLBCL as “advanced stage.”

How is DLBCL treated?

One of the most common treatments for diffuse large B-cell lymphoma is R-CHOP. It combines the monoclonal antibody drug rituximab with three chemotherapy drugs and a corticosteroid.

This treatment is safe and effective but doesn’t always work or keep DLBCL from coming back (recurring). Studies show relapses or recurrent cancer affect an estimated 30% to 40% of people with DLBCL who receive R-CHOP. If that happens, your provider may recommend the following treatments:

• Second-line therapy and autologous stem cell transplant. Second-line therapy is intensive treatment with combined cancer drugs.
• CAR T-cell therapy. This treatment is a type of immunotherapy that helps your T cells (a type of white blood cell) fight cancer more effectively.
• Targeted therapy. This treatment targets the genetic changes or mutations that cause diffuse large B-cell lymphoma.

Can diffuse large B-cell lymphoma be prevented?

There’s no guaranteed way to prevent DLBCL and nothing you can do about risk factors you can’t control, like having an immunodeficiency. But you can take steps to reduce your risk of developing certain infections linked to DLBCL, like hepatitis and HIV. You can also work to maintain a BMI (body mass index) that’s healthy for you.

What can I expect if I have DLBCL?

While some DLBCL can be life-threatening and difficult to treat, healthcare providers can often cure it using a combination of cancer drugs. Often, front-line or initial treatment sends DLBCL into complete remission. This means there are no signs and symptoms of cancer. Front-line treatment cures about 60% of people with DLBCL. 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.

According to the National Cancer Institute, 64.7% of all people with DLBCL are alive five years after diagnosis. Like many kinds of cancer, survival rates increase for people with early-stage cancer.

Still, while cure rates and survival rates may be helpful, your experience depends on many factors, including the type of DLBCL, cancer stage and your overall health. These are factors that only your healthcare provider knows about. Your provider is your best resource for answering questions about what to expect.

How do I take care of myself?

It can be stressful and exhausting to live with any form of cancer, including diffuse large B-cell lymphoma. It’s important that you do what you can to take care of yourself throughout DLBCL treatment. Here are some suggestions:

• Focus on nutrition. Know what foods to eat and avoid, so you’re nourishing your body.
• Rest. Fatigue is the most common side effect of DLBCL treatment. 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 health and check how the treatment is working.

Afterward, 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 the cancer has recurred.

When should I go to the emergency room?

Cancer treatments for DLBCL, including chemotherapy and immunotherapy, may have strong side effects. Your healthcare provider will advise you on how to manage them and prescribe medications to help as needed. Still, 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 my healthcare provider about DLBCL?

Questions you may want to ask include:

• What type of diffuse large B-cell lymphoma do I have?
• What’s this cancer’s stage?
• What treatments will I need?
• What treatment side effects should I expect?
• Will this treatment cure the cancer?
• What treatments are available if the cancer comes back?
• Can you refer me to a support group for people who have this kind of cancer?