What is B-Cell Lymphoma?

B-cell lymphoma is a form of cancer that starts in a white B-cell called a lymphocyte. B-cell lymphocytes make antibodies, the proteins in the immune system that help fight infections. They are often found in lymph nodes or other lymphoid tissues such as the spleen.

In B-cell lymphoma, some lymphocytes are no longer healthy and do not fight infection. Instead, they grow out of control, crowding out the normal cells and causing the lymph nodes to get bigger. As the disease advances, it may spread to the bone marrow, central nervous system, liver, spleen and reproductive organs.

Most B-cell lymphomas are non-Hodgkin lymphomas (NHL) and others are Hodgkin lymphomas. B-cell lymphomas are rare in children and adolescents but usually respond well to treatment.

The most common types of B-cell lymphomas in children are:

• B-cell lymphoblastic lymphoma — This cancer begins in the bone marrow, the spongy center of bones where new blood cells grow.
• Burkitt lymphoma — This fast-growing cancer accounts for about one-third of all lymphomas in children./li>
• Diffuse large B-cell lymphoma (DLBCL) — This aggressive, treatable cancer usually begins in the lymph nodes, which are part of the body’s immune system.
• Primary mediastinal B-cell lymphoma (PMBCL) — This cancer usually starts in the thymus, which is an organ in the upper chest.

B-Cell Lymphoma Causes

B-cell lymphoma develops when something changes the genetic code of a lymphocyte. DNA is what makes up the genetic code of all cells. This code tells the cells how to work. Changes to DNA change the genetic code of the cell. In the case of B-cell lymphoma, these changes make it impossible for the lymphocyte to fight infection. Instead, it just grows and divides.

In most children and young adults with B-cell lymphoma, the cause is unknown. However, people who have DNA repair defects or immune deficiencies, or those who take immunosuppressive medications, are at higher risk for developing this type of cancer.

B-Cell Lymphoma Symptoms

The symptoms of B-cell lymphoma can depend on what part of the body is affected by the disease, how fast it is growing and how far it has spread at the time of diagnosis.

A common symptom is painless swelling of the lymph nodes in the neck, armpit or groin. Other symptoms include:

• A swollen abdomen due to a mass or an enlarged liver or spleen
• Fatigue
• Cough or difficulty breathing
• Weight loss
• Fever

B-Cell Lymphoma Diagnosis

The first step in diagnosing B-cell lymphoma is to have a physical exam and blood work followed by imaging tests. Imaging tests can include:

• Chest X-ray to see if there are enlarged lymph nodes in the chest
• Computed tomography (CT) scan to check for lymphoma in the chest and abdomen
• Positron emission tomography (PET) scan to see if the disease has spread to the liver, bones, bone marrow or spleen

Doctors also take a biopsy of the mass or lymph node and examine the tissue under a microscope. They look for the presence, type and arrangement of cancer cells that are typical of the disease.

Some patients have a spinal tap (also called a lumbar puncture) to determine if the B-cell lymphoma has spread to the fluid that surrounds the brain and spinal cord.

These tests help the care team “stage” the B-cell lymphoma. When the cancer is diagnosed in stages 1-3, treatment is often shorter and less intense. When B-cell lymphoma is at stage 4, this means it has spread to the bone marrow or spinal fluid.

B-Cell Lymphoma Treatment

Good therapies exist for B-cell lymphoma, and the vast majority of children do very well. The therapy(s) chosen depend on the exact diagnosis and whether the disease has spread.

The standard treatment for B-cell lymphoma is chemotherapy. If the disease is located in one area of the body (localized), the treatment plan may include surgery and a short, less intensive course of chemotherapy. If the disease is more advanced, the treatment usually includes high-dose chemotherapy.

Some patients also need radiation therapy. Cincinnati Children’s is one of only a few children’s hospitals that offer proton therapy. This type of radiation therapy uses a highly precise proton beam. The beam destroys cancer cells while minimizing damage to surrounding healthy tissues and organs. As a result, patients have fewer short-term side effects or long-term complications of traditional radiation therapy.

Treatment Beyond Standard Therapies

About 90% of children and young adults with B-cell lymphoma are cured with standard therapies. But sometimes, B-cell lymphoma resists treatment or returns after a time of remission. When this happens, the care team at Cincinnati Children’s considers using intensive chemotherapy in combination with other therapies, such as:

• Hematopoietic stem cell transplantation (HSCT)
• Targeted B-cell agents
• Immunotherapy, including chimeric antigen receptor T-cell (CAR-T) therapy using Kymriah and Yescarta

Cincinnati Children’s is one of the first pediatric hospitals certified to provide CAR-T therapy.

Some of these therapies are available only through clinical trials. To learn more about available lymphoma clinical trials, contact us at cancer@cchmc.org or 513-636-2799.

B-Cell Lymphoma Prognosis

The overall survival rate for B-cell lymphoma in the United States is about 90%, depending on the type and stage of lymphoma. Children with localized disease have a better prognosis than those with advanced or widespread disease.

Cincinnati Children's is an international referral center for the treatment of children and young adults with high-risk and relapsed B-cell lymphoma. Our physicians lead national efforts in the development of new targeted therapies, immunological approaches and hematopoietic stem cell transplantation (HSCT).