Burkitt Lymphoma in Children

Burkitt lymphoma is a rare, fast-growing, aggressive form of non-Hodgkin mature B-cell lymphoma (NHL). It’s a type of cancer that starts in white blood cells in the lymphatic system. It's named for the doctor who first described it in children in Africa.

The lymphatic system is part of the immune system. It helps the body fight diseases and infections. It also helps balance fluids in different parts of the body. The lymphatic system includes:

• Lymph. This is a clear fluid that contains white blood cells called lymphocytes.
• Lymph vessels. These are tiny tubes that carry lymph fluid all over the body.
• Lymphocytes.  These are white blood cells that fight infections and disease. Burkitt lymphoma starts in the type of lymphocyte called mature B-cells. B-cells make antibodies to fight infections.
• Lymph nodes. These are small bean-shaped organs. They're scattered all over the body. They're connected by lymph vessels. And they filter the lymph fluid as it moves around the body.
• Other organs and body tissues. The lymphatic system includes the bone marrow where blood cells are made. It also includes the spleen, thymus, and tonsils.

There are 3 main types of Burkitt lymphoma in children:

• Endemic (may be called African Burkitt lymphoma).  Endemic means it's common in one area. This type is a common childhood cancer in central Africa, Brazil, and Papua New Guinea (along the equator). It causes large tumors of the face and starts in the jawbone and other facial bones. It's rare in the U.S.
• Sporadic (nonendemic). This is the type most often seen in the U.S., Canada, and Western Europe. It often causes large tumors in the belly (abdomen).
• Immunodeficiency-associated. This type mostly affects children who have a weak immune system due to HIV infection, an inherited disease, or because they take medicines after organ transplant. It also causes tumors on the abdomen.

In children, sporadic Burkitt lymphoma is most common in boys between ages 5 and 14 in the U.S.

Like other types of NHL, the exact cause of Burkitt lymphoma isn't known. Some viral infections may increase a child’s risk of having Burkitt lymphoma. The viruses that are linked to Burkitt lymphoma include:

• Epstein-Barr virus, the virus that causes mono (mononucleosis)
• HIV, the virus that causes AIDS
• In the U.S., Burkitt lymphoma often has genetic changes.

The symptoms of Burkitt lymphoma often start suddenly, and the tumors tend to grow very quickly. A child can become very sick in a few days to a few weeks.

Symptoms of a belly (abdominal) tumor can include:

• Abdominal pain and swelling
• Nausea and vomiting
• Trouble with bowel movements (constipation)
• Poor appetite
• Feeling full quickly, such as after eating a small amount of food

Other symptoms may include:

• Painless swelling of the lymph nodes in the neck, chest, abdomen, underarm, or groin
• Fever
• Chills
• Night sweats
• Tiring easily (fatigue)
• Weight loss
• Itchy skin
• Coughing or trouble breathing

The symptoms of Burkitt lymphoma can look like other health conditions. Make sure your child sees a healthcare provider for a diagnosis.

Your child's healthcare provider will ask about your child's health history and symptoms. They will examine your child. Your child may need tests, such as:

• Blood and urine tests. Blood and urine samples are tested in a lab for signs of cancer and to check organ function. HIV testing may be done.
• Tissue biopsy. A small piece of tissue (called a sample) is taken from the tumor, lymph nodes, or other tissue. It’s sent to a lab and tested for cancer cells.
• Chest X-ray. This shows the heart, lungs, and other parts of the chest. 
• CT (computed tomography) scan. This may be done to look at the abdomen, chest, and pelvis. A CT scan uses a series of X-rays taken from different angles and a computer to make detailed 3-D pictures of the inside of the body. Your child may drink a contrast medium (may be called a dye). Or contrast may be injected into a vein before having the scan. The contrast helps show more details.
• MRI (magnetic resonance imaging) scan. An MRI uses large powerful magnets, radio waves, and a computer to make detailed pictures of the inside of the body. This test might be used to check the brain and spinal cord. Or it may be used if the results of an X-ray or CT scan are unclear. A contrast medium may be injected into a vein before having the scan.
• PET (positron emission tomography) scan. This scan is used to look for active tumor cells all over the body. A small amount of radioactive glucose is injected into a vein. Sometimes a PET scan and CT scan are done at the same time (PET/CT scan).
• Ultrasound. This is also called sonography. High-energy sound waves and a computer are used to make pictures of blood vessels, tissues, and organs.
• Bone marrow aspiration or biopsy. Bone marrow is the thick liquid in the center of some bones. It’s where blood cells are made. A small amount of bone marrow fluid may be taken out. This is called aspiration. Or solid pieces of bone marrow tissue may be taken. This is called a core biopsy. Bone marrow is often taken from the back of the hip bone. This test may be done to see if cancer cells have spread to the bone marrow.
• Lumbar puncture (spinal tap). A thin needle is put between the bones in the lower back and into the spinal canal. This is the area around the spinal cord. This is done to see if there are cancer cells in the brain and spinal cord. A small amount of cerebrospinal fluid (CSF) is taken out and sent for testing. CSF is the fluid around the brain and spinal cord.

Part of diagnosing cancer is called staging. Other tests may be recommended for staging and before treatment. Staging is the process of seeing if the cancer has spread, and where it has spread. Staging also helps to decide on the best treatment. There are different ways of staging NHL. But most range from stage 1 to stage 4. Stage 4 is cancer that has spread to parts of the body that are not part of the lymphatic system. Talk with your child's healthcare provider about the stage of your child's cancer and what it means.

Treatment will depend on the type and stage. Burkitt lymphoma is most often treated with:

• Chemotherapy (chemo). These are strong medicines that kill cancer cells or stop them from growing. Chemo is the main treatment for Burkitt lymphoma. Often more than one type of chemo is used. If cancer cells are found in the CSF, chemo will need to be given there, too.
• Immunotherapy. These are medicines that boost the immune system to better find and kill cancer cells. They're often given along with chemo. Rituximab is one of the medicines that may be used. Other immunotherapy medicines are being researched.
• Surgery. If there's only one large tumor, surgery may be done to remove it. This is done before chemotherapy.
• Clinical trials. Most children with cancer are treated as part of a clinical trial. Taking part in a clinical trial means your child gets the best treatment available today, and might also get new treatments that are thought to be even better. Before starting treatment, talk to your child's healthcare provider to find out if there are any treatments being tested that may work well for your child. Many new treatments are only available in clinical trials.

Burkitt lymphoma grows quickly and the tumors are often very big. When chemo starts, it kills a lot of cancer cells in a short time. This waste from the contents of the killed cancer cells can build up in your child's blood and cause a problem called tumor lysis syndrome (TLS). TLS can cause kidney damage and problems with the heart and nervous system. Your child will be given medicines and a lot of IV fluids to help keep this from happening.

Your child will need follow-up care during and after treatment to:

• Check on your child's response to the treatment
• Manage the side effects of treatment
• See if the cancer has come back or spread

With treatment, most children with Burkitt lymphoma go on to live long lives. With any cancer, how well a child is expected to recover (their prognosis) varies. Keep in mind:

• Getting medical treatment right away is important for the best prognosis. 
• Ongoing follow-up care during and after treatment is needed.
• New treatments are being tested to improve outcome and reduce side effects.
• Talk with the healthcare provider about any concerns you may have or problems you may notice. Your child's treatment team wants to know as much as they can about how your child is doing.

Possible complications depend on the type and stage of the lymphoma, as well as the treatment used. Your child may have short- and long-term problems from the tumor or from treatment. These may include things like:

• Increased risk for infection
• Increased risk for bleeding
• Nausea and vomiting
• Diarrhea
• Poor appetite
• Sores in the mouth
• Hair loss
• Heart disease
• Lung problems
• Increased risk for having other cancers later in life
• Trouble having children (infertility)
• Tumor lysis syndrome

Many chemo side effects can be treated to keep them from getting worse. There may even be things you can do to help prevent some of them. Talk with the healthcare provider about what you should watch for and what can be done to help prevent complications.

You can help your child manage their treatment in many ways. For example:

• Your child may have trouble eating. A dietitian may be able to help.
• Your child may be very tired. They will need to balance rest and activity. Encourage your child to get some exercise. This is good for overall health. And it may help to reduce tiredness.
• Get emotional support for your child. Find a counselor, psychologist, or child support group that can help.
• Make sure your child goes to all follow-up appointments.

Call the healthcare provider if your child has:

• Symptoms that get worse
• New symptoms
• Side effects from treatment that affect your child's daily function or don't get better with treatment

Ask your child's healthcare provider what signs to watch for and when to call. Know how to get help after office hours and on weekends and holidays.

• Burkitt lymphoma is a very fast-growing, aggressive type of non-Hodgkin lymphoma.
• It often causes large belly (abdominal) tumors.
• A child may have swollen lymph nodes, tiredness, fever, and other symptoms.
• It's treated mainly with chemotherapy and possibly with surgery.
• With treatment, most children with Burkitt lymphoma go on to live long lives.
• Ongoing care after treatment is important.

Tips to help you get the most from a visit to your child’s healthcare provider:

• Know the reason for the visit and what you want to happen.
• Before your visit, write down questions you want answered.
• At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.
• Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.
• Ask if your child’s condition can be treated in other ways.
• Know why a test or procedure is recommended and what the results could mean.
• Know what to expect if your child does not take the medicine or have the test or procedure.
• If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
• Know how you can contact your child’s healthcare provider after office hours. This is important if your child becomes ill and you have questions or need advice.