Burkitt Lymphoma in Children

Burkitt lymphoma is a rare, fast-growing form of non-Hodgkin lymphoma (NHL). It’s a type of cancer that starts in white blood cells in the lymphatic system. The lymphatic system is part of the immune system. It helps to fight diseases and infections. The lymphatic system 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 a type of white blood cell that fights infections and disease. Burkitt lymphoma starts in one type of lymphocyte called B-cells.
• 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 is made. It also includes the spleen, thymus, tonsils, and digestive tract.

There are 3 main types of Burkitt lymphoma:

• Endemic (African).  Endemic means it is common in one area. This type is a common childhood cancer in central Africa and New Guinea. It causes large tumors of the face and jaw. It's rare in the U.S.
• Sporadic (nonendemic).  This is the type most often seen in the U.S. and Western Europe. It often causes large tumors in the belly (abdomen).
• Immunodeficiency-associated. This type mostly affects people with HIV infection. The lymph nodes, bone marrow, and brain and spinal cord (central nervous system) are often affected.

In children, Burkitt lymphoma is most common in boys between ages 5 and 10.

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 (EBV), the virus that causes mono (mononucleosis)
• HIV, the virus that causes AIDS

The symptoms of Burkitt lymphoma 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. He or she will examine your child. Your child may need tests such as:

• Blood and urine tests. Blood and urine samples are tested in a lab.
• Tissue biopsy.  A small tissue sample is taken from the tumor, lymph nodes, or other tissue. It’s checked with a microscope for cancer cells.
• Chest X-ray . This shows the heart, lungs, and other parts of the chest. 
• CT scan. This may be done to look at the abdomen, chest, and pelvis. A CT scan uses a series of X-rays and a computer to make detailed pictures of the inside of the body. Your child may drink a contrast dye. Or a dye may be injected into a vein. The dye helps show more details.
• MRI scan.  An MRI uses large magnets, radio waves, and a computer to make detailed pictures of the inside of the body. This test is 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. 
• Ultrasound. This is also called sonography. Sound waves and a computer are used to make pictures of blood vessels, tissues, and organs.
• Bone marrow aspiration or biopsy.  Bone marrow is found 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 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 reached the bone marrow.
• Lumbar puncture (spinal tap).  A thin needle is placed 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 cerebral spinal 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. 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 often treated with:

• Chemotherapy medicines (chemo). These are medicines that kill cancer cells or stop them from growing. This 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 in that area too.
• Surgery. If there's only one large tumor, surgery may be done to remove it. This is done before chemotherapy.
• Clinical trials.  Ask your child's healthcare provider 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. These dead cells can build up in your child's body 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 cancer has returned 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 (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 to lessen side effects.
• Talk with the doctor 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. They may include things like:

• Increased risk for infection
• Increased risk for bleeding
• Heart disease
• Lung problems
• Increased risk for having other cancers later in life
• Trouble having children (infertility)
• Death

• Nausea and vomiting
• Diarrhea
• Poor appetite
• Sores in the mouth
• Hair loss

Talk with the doctor about what you should watch for and what can be done to help prevent complications.

You can help your child manage his or her 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. He or she 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 lessen tiredness.
• Get emotional support for your child. Find a counselor 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

• Burkitt lymphoma is a very fast-growing 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 with chemotherapy and surgery.
• With treatment, most children with Burkitt lymphoma go on to live long lives.
• Ongoing care 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 provider after office hours. This is important if your child becomes ill and you have questions or need advice.