What is AIDS-related lymphoma in children?

AIDS-related lymphoma is a type of cancer called non-Hodgkin lymphoma (NHL). Children with AIDS have a higher risk for this cancer. AIDS is a disease that makes the immune system weak. It raises the risk for infection and some kinds of cancer, like NHL.

AIDS-related lymphoma starts in white blood cells in the lymph system. The lymph system is part of the immune system. It helps fight diseases and infections. It also helps balance fluids in different parts of the body. The lymph system includes:

• Lymph. This is a clear fluid that contains white blood cells called lymphocytes.

• Lymph vessels. This network of tiny tubes carries lymph fluid all through the body.

• Lymphocytes. These are a type of white blood cell that fight infections and disease. There are T and B lymphocytes.

• Lymph nodes.  These small bean-shaped organs are found along the lymph vessels. They filter the lymph fluid as it moves around the body. Groups of them can be found in places like the arm pit, neck, groin, pelvis, and chest.

• Other organs and tissues. The lymph system includes the bone marrow, which is where blood cells are made. It also includes the spleen, thymus, and tonsils. Lymph tissue can be found in the digestive tract, skin, thyroid, and brain, too.

The lymph system includes many parts of the body, so AIDS-related lymphoma can start almost anywhere and affect any organ. Along with the lymph system, it commonly affects the brain and spinal cord (central nervous system) and the lining of body cavities. These include the chest, the belly (abdomen), and the sac containing the heart (pericardium).

The 2 main types of AIDS-related lymphoma are:

• Diffuse large B-cell lymphoma

• Burkitt or Burkitt-like lymphoma

Both grow very fast and can grow outside the lymphatic system.

What causes AIDS-related lymphoma in a child?

HIV is the virus that causes AIDS. People are often infected with HIV through contact with the blood or other body fluids of someone with HIV or AIDS. In children, this contact often happens during pregnancy, labor, and delivery, or through breastfeeding. It happens when a birth parent has HIV or AIDS and is not taking antiviral medicine to help decrease the risk of HIV transmission.  

The HIV virus attacks the body's immune system. This makes it harder for the immune system to fight cancers and infections. People with AIDS have an increased chance of lymphoma and other types of cancer. They're also at a high risk for infections.

Which children are at risk for AIDS-related lymphoma?

Children born to birth parents who are not taking antiviral medicines to control HIV are at high risk for AIDS-related lymphoma.

But all people with HIV or AIDS, no matter their age, are at risk for AIDS-related lymphoma.

What are the symptoms of AIDS-related lymphoma in a child?

Your child may have many different symptoms. It depends on the type of lymphoma and where it is. Symptoms may include:

• Swollen, painless lymph nodes in the neck, belly (abdomen), underarm, or groin

• Trouble breathing

• Loud breathing or wheezing

• Drenching night sweats

• Unexplained weight loss

• Fever with no known reason

• Trouble swallowing

• Head or neck swelling

• Belly swelling and pain from an enlarged spleen or liver

• Extreme tiredness (fatigue)

Many of these symptoms can be caused by other health problems. It's important to take your child to a healthcare provider if you notice these symptoms. Only a healthcare provider can tell if your child has cancer.

How is AIDS-related lymphoma diagnosed in a child?

If your child has HIV, they will be closely watched for health problems. This includes changes that might be signs of AIDS-related lymphoma. 

Your child's healthcare provider will ask about your child's health history and symptoms. A physical exam will be done. Your child may need tests, such as:

• Blood and urine tests. Blood and urine samples are sent to a lab to be checked for signs of diseases, like HIV, infection. or cancer. These tests also check organ function.

• Tissue and lymph node biopsy.  Tiny pieces of tissue (called samples) are taken from the lymph nodes or other body tissue. They’re sent to a lab and checked for cancer cells. There are many ways to do a biopsy. Your child's provider will talk with you about the best option based on where the lymphoma is. A biopsy is the only way to know for sure if your child has lymphoma and what type it is. If cancer is found, several tests may be done to study the cancer cells closely. This is to check for certain genes, proteins, and other factors.

• Chest X-ray. This shows the heart, lungs, and other parts of the chest.

• CT scan. This 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 dye (contrast medium). Or it may be put into a vein. The contrast helps show more details.

• MRI scan. An MRI uses large powerful magnets, radio waves, and a computer to make detailed pictures of the inside of the body. A contrast dye may be injected into your child's vein. It helps show details more clearly. This test is often used to check the brain and spinal cord. Or it may be used if the results of an X-ray or CT scan are not clear. 

• Ultrasound. This is also called sonography. High-energy sound waves and a computer are used to make pictures of lymph nodes, blood vessels, tissues, and organs.

• PET (positron emission tomography) scan. A small amount of radioactive sugar (glucose) is put into a vein. Pictures are taken all over the body where this glucose is being used. Cancer cells show up brighter on this scan. This is because they are more active and take up more glucose than normal cells. The PET scan may be combined with a CT scan called a PET-CT scan.

• 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 can be taken out. This is called aspiration. Or solid piece of bone marrow tissue may be removed. This is called a core biopsy. Bone marrow is most often taken from the back of the hip bone. This test may be needed to see if cancer cells have reached the bone marrow.

• Lumbar puncture (spinal tap).  A thin needle is put between 2 bones of the low back and into the spinal canal. This is the area around the spinal cord. A lumbar puncture 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 that flows around the brain and spinal cord.

• Pleural or peritoneal fluid sampling.  Fluid is removed from around the lungs (pleural) or the belly (peritoneal). It's then checked for lymphoma cells.

Medicine might be used to make your child sleep and not feel pain for some of these tests.

Part of diagnosing cancer is called staging. Staging is the process of finding out how much cancer there is and how far it has spread (metastasized) in your child's body. It's one of the most important things to know when deciding how to treat the cancer.

There are different staging systems used for NHL. But most range from stage 1 to stage 4. Stage 4 is lymphoma that has spread to the brain or spinal cord or the bone marrow when it's first found. Talk with your child's healthcare provider about the stage of your child's cancer and what it means.

How is AIDS-related lymphoma treated in a child?

Treatment will depend on the type and stage of lymphoma. It will also depend on:

• When your child first had treatment for HIV/AIDS

• If the cancer has spread to the brain and spinal cord and/or bone marrow

• If there are certain gene changes in the lymphoma cells

Treatment may include any of the below:

• Chemotherapy (chemo).  These are strong medicines that kill cancer cells or stop them from growing. This is the main treatment for lymphoma. 

• Radiation therapy.  These are high-energy X-rays or other types of radiation used to kill cancer cells. Radiation isn't used a lot to treat children with cancer. Still, it might be used if lymphoma has spread to the CSF. Or it may be used to treat cancers that are causing problems, such as pressing on nerves and causing pain or making it hard to breathe by pressing on the breathing tubes.

• Monoclonal antibodies.  This is a type of targeted drug therapy that uses immune system proteins made in the laboratory. Monoclonal antibodies focus on and kill the cancer cells or keep them from growing and spreading. It causes less harm to healthy cells.

• High-dose chemotherapy with a stem cell transplant.  Young blood cells (called stem cells) are taken from the child or from someone else (a donor). Then high doses of chemo are given. This damages the bone marrow. After the chemo, the stem cells are put into the child's blood to replace the bone marrow and, over time, make healthy blood cells.

• Supportive care. Treatment can cause side effects. Supportive care is medicine and other treatments used for pain, fever, infection, and nausea and vomiting.

• 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.

Managing the HIV infection itself is also a key part of treating AIDS-related lymphoma. Your child will be given highly active antiretroviral treatment to control the virus.

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

• Keep the HIV under control

What are the side effects of AIDS-related lymphoma treatment in a child?

Cancer treatments, like chemotherapy and radiation, can damage normal cells. This can cause side effects. Possible side effects depend on the type and stage of the lymphoma, as well as the type or types of treatments used. Common side effects can include:

• Nausea and vomiting

• Diarrhea

• Poor appetite

• Sores in the mouth

• Hair loss

• Increased risk of infection

• Easy bleeding and bruising

• Heart problems

• Lung problems

• Increased chance of having other cancers later in life

• Trouble having a baby (infertility)

Many chemo side effects can be treated to keep them from getting worse. There may even be things you can to do help prevent some of them. Most side effects go away over time after treatment ends. But some may not start until a long time after treatment ends. Talk with your child's healthcare provider about what you should watch for. Also ask what can be done to help prevent or treat side effects.

How can I help my child live with AIDS-related lymphoma?

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

• 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 a child support group that can help.

• Make sure your child goes to all follow-up appointments.

• Protect your child from infections. Have your child wash their hands often. Stay away from people who are sick.

• Call your child's healthcare provider if your child has any signs of infection, including fever.

When should I call my child’s healthcare provider?

Your child's healthcare provider will talk with you about when to call. You may be told to call if your child has:

• New symptoms or symptoms that get worse

• Signs of infection, such as fever

• Side effects of treatment that affect your child's daily activities or don't get better with treatment

Ask the provider what signs to watch for and when to call. Know how to get help after office hours and on weekends and holidays.

Key points about AIDS-related lymphoma in a child

• AIDS-related lymphoma is a type of non-Hodgkin lymphoma. 

• It may affect the lymph system, brain and spinal cord, and many other parts of the body.

• Treatment depends on the type, stage, and other factors. It may include chemotherapy, radiation therapy, targeted therapy, and/or stem-cell transplant.

• Treating the HIV is an important of your child's health.

Next steps

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, and on weekends and holidays. This is important if your child becomes ill or you have questions or need advice.