Diffuse Large B-Cell Lymphoma (DLBCL)

What is diffuse large B-cell lymphoma?

Symptoms of diffuse large B-cell lymphoma

Symptoms of DLBCL may include:

• Swollen lymph nodes in the neck, underarm, groin, or abdomen
• Fever
• Night sweats
• Weight loss
• Coughing
• Trouble breathing
• Swollen belly and/or belly pain
• Loss of appetite
• Nausea
• Vomiting
• Fatigue

Risk factors for diffuse large B-cell lymphoma

Risk factors may increase your child’s chances of getting a disease. Little is known about the risk factors for DLBCL in children. For lymphoma, the risk factors we know so far are:

• Weak immune system after a bone marrow or solid organ transplant
• Infection with Epstein-Barr virus or human immunodeficiency virus (HIV)
• A genetic disorder, such as ataxia-telangiectasia, that increases the risk of having cancer
• More common in males than females
• More common in White than Black children

Diagnosis of diffuse large B-cell lymphoma

A cancer diagnosis requires tests and procedures. The doctor will give your child a physical exam, ask about their medical history, and order tests such as:

• Biopsy
• Bone marrow aspiration and biopsy
• Imaging tests
• Ultrasound
• Blood tests: complete blood count, blood chemistry, lactate dehydrogenase, metabolic panel, and/or uric acid
• HIV or EBV test

See Waiting for Test Results.

If the pathologist finds lymphoma cells in the biopsy sample, the lab will do more lab tests on the sample, such as flow cytometry, immunophenotyping, and cytogenetic analysis, if they are available.

There are several types of DLBCL:

• Primary mediastinal B-cell lymphoma, which grows quickly in the chest
• CNS lymphoma, which grows in the central nervous system
• DLBCL-NOS, which has 2 types based on genetic changes:
  • Germinal center B-cell (GBC; most common in children)
  • Activated B-cell (ABC)

Your care team may order tests to find out which type of DLBCL your child has.

Stages of diffuse large B-cell lymphoma

The care team plans treatment based on the cancer stage. Some tumors grow rapidly. So, the doctor must stage the lymphoma as quickly as possible.

To find out the stage of disease, doctors may order more tests such as:

• Bone marrow aspiration and biopsy
• Lumbar puncture (spinal tap)
• PET/CT imaging

The stage will tell how much cancer is present and if it has spread to other parts of the body.

The International Pediatric Non-Hodgkin Lymphoma Staging System divides the disease into 4 stages:

• Stage 1 and 2 lymphomas are limited-stage disease and are treated the same way.
• Stage 3 and 4 lymphomas are advanced-stage disease. The treatment for both stages is similar, but the length of treatment may vary depending on the stage.

Treatment of diffuse large B-cell lymphoma

Treatment of DLBCL depends on:

• The type of lymphoma
• The stage of lymphoma
• How well the disease may respond to treatment
• Available treatments
• The patient’s age and health

Treatments may include:

• Chemotherapy
• Targeted therapy
• Immunotherapy
• Surgery to remove all or part of the tumor

Higher-stage, advanced disease is often treated with chemotherapy and immunotherapy. This is known as combination therapy. The most commonly used treatment in North America is the Lymphome Malin de Burkitt (LMB) treatment. It includes chemotherapy with or without the targeted therapy rituximab.

Treatment can sometimes cause side effects. One that may occur early in treatment is tumor lysis syndrome. This happens when cancer cells quickly die and break apart. The care team will watch your child closely for possible side effects. Ask the care team if you have questions.

Relapsed disease

A common chemotherapy plan for relapsed disease is RICE (rituximab, ifosfamide, carboplatin, and etoposide) followed by a stem cell transplant. New targeted therapies are being studied. Some patients may be able to take part in clinical trials to test new treatments.

Prognosis for diffuse large B-cell lymphoma

The prognosis depends on several factors, including the stage of the disease.

In the United States, the 5-year survival rate for limited-stage (stage 1 and 2) diffuse large B-cell lymphomas is higher than 95%. The survival rate for advanced-stage (stage 3 and 4) DLBCL is higher than 90%.

Support for patients with diffuse large B-cell lymphoma

Coping with a cancer diagnosis and treatment can be stressful for the patient and the family. Talking to a social worker, psychologist, or another mental health specialist may help.

Learn how to talk to your child about cancer.

After treatment, your care team may use imaging tests and exams to watch for recurrence.

Childhood cancer survivors should get follow-up care throughout their lives. Some treatments can cause late effects. These are health problems that happen months or years after treatment ends.

After completing treatment, it is important that your child:

• Has regular checkups and screenings by a primary care provider
• Maintains healthy habits to protect their health, including physical activity and healthy eating
• Has a survivorship care plan to share with their health providers, which includes:
  • Guidance on health screenings
  • Disease risk factors
  • Ways to improve health

Questions to ask your care team

• What does the biopsy show?
• What stage of cancer does my child have?
• What are my child’s treatment options?
  
• What are the possible side effects of each treatment?
• How can my child manage their side effects?
• Will my child need to be in the hospital for treatment?
• Where is the treatment available? Is it close to home or will we have to travel?
• What resources are available to help us cope with this illness?
• What monitoring does my child need after treatment?
• How can I help my child stay healthy?
• What are the possible late effects of treatment?

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