Diffuse Large B Cell Lymphoma (DLBCL)

August 15, 2024

This page was reviewed under our medical and editorial policy by Alex Herrera, M.D., associate professor, Division of Lymphoma, Department of Hematology & Hematopoietic Cell Transplantation, City of Hope® Cancer Center Duarte

Lymphoma is a type of cancer that affects the lymph nodes — small structures scattered throughout the body that are part of the immune system. Lymphoma has many forms. Diffuse large B cell lymphoma (DLBCL), a type of non-Hodgkin lymphoma, is the most common.

Here’s what patients and their families should know about the diagnosis and treatment of diffuse large B cell lymphoma.

What Is Diffuse Large B Cell Lymphoma?

B cell lymphomas, such as DLBCL, affect the body’s B lymphocytes. These are a type of white blood cell that makes antibodies to help the immune system fight infections. They originate from stem cells in a person’s bone marrow.

Diffuse large B cell lymphoma accounts for 1 in every 3 cases of lymphoma. While it is typically an aggressive and fast-growing cancer, DLBCL tends to respond well to treatment.

Diffuse large B cell lymphoma may be categorized into a number of subtypes. These subtypes may have different treatments or prognoses (a gauge of how well a patient will do with treatment), but not all cases of DLBCL have a subtype.

Diffuse Large B Cell Lymphoma Symptoms

Often, the first sign of diffuse large B cell lymphoma that a patient notices is a rapid swelling in the neck, armpits or groin caused by enlarged lymph nodes. For many patients, this swelling will be painless, but some may experience pain depending on the location.

Other symptoms of DLBCL may include:

  • Fever
  • Soaking sweats at night
  • Extreme tiredness that does not get better with rest
  • Loss of appetite
  • Unexplained weight loss
  • Pain
  • Shortness of breath

Since non-Hodgkin lymphomas like DLBCL spread quickly through the bloodstream, many patients do not experience symptoms that prompt them to seek medical attention until the disease has reached an advanced stage.

What Causes Diffuse Large B Cell Lymphoma?

Diffuse large B cell lymphoma starts with changes to the genes in developing B cells, part of the body’s immune system. Researchers have found that B cells develop in three stages: pre-germinal, germinal and post-germinal center. Most of the time, DLBCL comes from cells in the middle stage of their growth.

Several causes may underlie these gene changes. These include mutations in a gene known as BCL6, certain medical conditions or exposure to certain types of chemicals. In addition, other types of lymphoma may transition into DLBCL over time.

No matter the initial cause, the changes in the B cell genes disrupt their normal life cycle. When this happens, they may begin to grow and divide uncontrollably, eventually becoming cancerous.

DLBCL Risk Factors

While anyone may develop diffuse large B cell lymphoma, it is more likely to occur in some people than in others. Some risk factors for DLBCL may include:

  • A personal history of HIV/AIDS or Epstein-Barr virus
  • Taking anti-rejection medicines to protect an organ transplant
  • Being diagnosed with another type of lymphoma or chronic lymphocytic leukemia
  • A family history of lymphoma
  • Having had radiation therapy or chemotherapy in the past
  • Exposure to chemicals such as dyes or pesticides
  • An underlying autoimmune disease

Additionally, DLBCL occurs more often in white males than in other groups. The median age at diagnosis is 64 years, meaning half of patients are diagnosed younger, half are older. The risk rises with age.

DLBCL Diagnosis

If a patient’s symptoms or a physical exam point to diffuse large B cell lymphoma, the doctor will perform a biopsy to obtain a sample of an affected lymph node for analysis. This is a surgical procedure that may be performed using local or general anesthesia. The cells taken from the sample are then examined under a microscope for cancer.

Once a diagnosis of diffuse large B cell lymphoma is confirmed, the medical team may perform additional tests to determine the subtype of DLBCL (if any) and the cancer’s stage. With this information, they then recommend a customized treatment plan.

Additional tests may include:

  • Blood tests
  • A spinal tap (if there is concern that the central nervous system is affected)
  • Positron emission tomography (PET) and computed tomography (CT) scans to determine the extent of the cancer

Diffuse Large B Cell Lymphoma Subtypes

Some cases of DLBCL are classified into subtypes based on their various characteristics. Some of the subtypes of diffuse large B cell lymphoma include those below.

Primary mediastinal B cell lymphoma: This subtype occurs most often in younger women. It starts in the middle of the chest, behind the breastbone, and it may cause breathing issues and swelling of the arms and face without treatment.

Double-hit or triple-hit lymphoma: These subtypes have changes to two (double) or three (triple) specific genes in the cancer cells. These cell changes are detected using advanced lab tests and do not cause symptoms different from those of DLBCL.

Other possible subtypes include primary DLBCL of the central nervous system, primary cutaneous DLBCL leg type or DLBCL associated with chronic inflammation.

Sometimes, other subtypes of non-Hodgkin lymphoma (such as follicular lymphoma) can transform to become diffuse large B-cell lymphoma (transformed lymphoma).

When a patient does not fall into a specific subtype, it is referred to as diffuse large B cell lymphoma, not otherwise specified (DLBCL-NOS).

Diffuse Large B Cell Lymphoma Stages

A cancer’s stage refers to how much cancer is in the body and whether it has spread. Categorizing cancers by stage helps medical providers plan the most appropriate treatments, determine a prognosis and identify potential clinical trials.

Several different cancer staging systems are in use. The one used for lymphoma staging is based on a system called the Lugano classification. The stages in this system are listed below.

Stage 1: Only one lymph node region or site is involved.

Stage 1E: Lymphoma has spread to one area outside the lymph node.

Stage 2: Lymphoma has spread to two or more lymph nodes on the same side of the diaphragm.

Stage 2E: Lymphoma is in a group of lymph nodes and in one area of a nearby organ, and sometimes also affects other lymph node groups on the same side as the diaphragm.

Stage 3: Lymph nodes on both sides of the diaphragm are involved.

Stage 4: Lymphoma has spread to one or more organs outside the lymphatic system, such as the bone marrow, liver or lung.

DLBCL Treatment

Lymphoma treatment is a rapidly evolving area of medicine, with new advances emerging all the time. During DLBCL treatment, patients will receive care from a multidisciplinary team that may include oncologists, physician assistants, nurses, social workers, physical therapists and other experts depending on individual needs.

The most common treatments for diffuse large B cell lymphoma include:

  • Chemotherapy, which uses medications to destroy cancer cells
  • Radiation therapy, which uses high-energy X-rays or protons to destroy cancer cells
  • Monoclonal antibodies, a type of therapy that stimulates the immune system to target cancer cells
  • Chimeric antigen receptor (CAR) T cell therapy, a therapy that uses the patient’s own T cells to target and destroy lymphoma cells
  • Immunotherapy: Immunotherapy treatment, such as the use of bispecific antibodies, is used to give the patient’s own immune system a boost or relies on engineered versions of normal parts of the immune system to assist.
  • Targeted therapy: Targeted therapy uses drugs designed to target parts of cancer cells. The drugs may block signals to multiply or turn on signals telling target areas to destroy themselves.

Diffuse Large B Cell Lymphoma Survival Rate

DLBCL survival rates are estimates of the percentage of people with the same type and stage of cancer who are still alive five years after their diagnosis. These estimates are based on information about a large number of people in the past. While they offer an indication of how successful treatment might be, they are unable to predict any individual patient’s course.

Based on people diagnosed with diffuse large B cell lymphoma between 2012 and 2018, the overall five-year relative survival rate is 65%, according to the American Cancer Society. For those with earlier, localized lymphoma, the survival rate is 73%.

It is important to remember that this rate may be affected by individual factors, such as the patient’s age, other health conditions or how well they tolerate treatments.

Additionally, treatments available for DLBCL are constantly evolving and have improved over time. This means that people diagnosed today may have more successful treatment options available than patients diagnosed five to 10 years ago.

References
References
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