Pediatric Non-Hodgkin Lymphoma

Non-Hodgkin lymphoma is cancer that starts in the lymphatic system. The lymphatic system is part of the immune system. It helps to fight diseases and infections. 

NHL results when the cells in the lymphatic system (lymphocytes) are changed (mutated) and grow out of control. These cells can spread to other organs and tissues in the body (metastasize). NHL is rare in children. But it can happen at any age. It tends to affect boys more often than girls. 

There are different types of NHL in children. But most tend to grow quickly. Your child's treatment team may order different tests based on what type of lymphoma is found. Treatment choices also depend on the specific type of lymphoma.

Researchers don't know the exact cause of non-Hodgkin lymphoma. Inherited gene changes and some viral infections may increase a child’s risk of having NHL. Risk factors that might be linked to NHL include:                  

• Epstein-Barr virus infection, the virus causes mono (mononucleosis)
• Infection with HIV, the virus that causes AIDS
• Past cancer treatment
• Certain immune diseases passed on in families (inherited syndromes).
  Taking anti-rejection medicines after organ transplant

In many cases, non-Hodgkin lymphoma in children may not cause symptoms until it has grown or spread. Many children have advanced disease at the time of diagnosis. This is because the symptoms start suddenly, and the tumors tend to grow fast. A child can become very sick in a very short time (a few days or weeks).

Signs and symptoms depend on the type of NHL and where it starts.

Symptoms of a belly (abdominal) tumor can include:

• Abdominal pain
• Nausea and vomiting
• Fever
• Trouble with bowel movements (constipation)
• Poor appetite
• Feeling full after eating only a small amount.

Symptoms of a chest tumor include:

• Trouble breathing or swallowing
• Pain with deep breaths
• Cough or wheezing
• High-pitched breathing sounds
• Swelling or skin looks blue on the head and arms 

Other symptoms may include:

• Painless swelling of the lymph nodes in neck, chest, abdomen, underarm or groin
• Fever for no known reason
• Sore throat
• Headaches
• Bone and joint pain
• Night sweats that soak pajamas and bedding
• Chills
• Tiring easily (fatigue)
• Weight loss
• Poor appetite
• Itching of the skin
• Recurring infections
• Low blood cell counts

The symptoms of NHL can be like those of many other health problems. Make sure your child sees a health care provider for a diagnosis.

Your child's health care provider will ask about your child's health history, family history and symptoms. A physical exam will be done. Based on the findings, your child may need tests, such as:

• Blood and urine tests. Blood and urine are collected and tested in a lab.
• Lymph node biopsy. A tiny piece of tissue, called a sample, is taken from the lymph nodes (or other abnormal areas). This is often done during surgery. The sample is tested for cancer cells. This type of biopsy is needed to diagnose NHL.
• Chest X-ray. A chest X-ray shows the heart, lungs, and other parts of the chest. It can show NHL that has spread to lymph nodes in 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. Sometimes a contrast substance is put in the blood to get clearer pictures.
• MRI scan. An MRI uses large magnets, radio waves, and a computer to make detailed pictures of the inside of the body. This test can 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. 
  
• Ultrasound. This is also called sonography. Sound waves and a computer are used to make pictures of blood vessels, tissues and organs.
• PET scan. For this test, a radioactive sugar is put into the blood. Cancer cells use the sugar faster than normal cells, so the sugar will collect in cancer cells. A special camera is then used to scan the whole body to see where the radioactive sugar has collected. A PET scan can sometimes spot cancer in different parts of the body, even when they can’t be seen by other tests. This test is often used together with a CT scan. This is called a PET/CT scan.
• Bone marrow aspiration or biopsy. Bone marrow is a thick liquid found in the center of some bones. It’s where blood cells are made. A small amount of bone marrow may be removed with a needle. This is called aspiration. Sometimes solid bone marrow tissue is taken with a bigger needle. This is called a core biopsy. Bone marrow is usually removed from the back of the hip bones. This test is done to see if there are cancer cells in the bone marrow.
• Lumbar puncture. A long, thin needle is put into the lower back, between the bones of the spine, and into the spinal canal. This is the area around the spinal cord and brain that contains fluid, called cerebral spinal fluid or CSF. CSF cushions and supports the spinal cord and brain. A small amount of CSF is removed and tested for cancer cells.
• Pleural or peritoneal fluid sampling. Fluid is removed from around the lungs (pleura) or abdomen (peritoneum). The fluid is checked for cancer cells.    

Part of diagnosing cancer is called staging. Staging is the process of finding out how much cancer there is and if it has spread. Staging is used to decide the best treatment choices.

There are different ways of staging NHL. Talk with your child's health care provider about the stage of your child's cancer and what it means. The system most commonly used divides NHL into four stages:

• Stage I (1). The lymphoma is in only 1 place. It’s either in lymph nodes in only 1 part of the body or is only 1 tumor that's not in a lymph node. Stage I NHL is not in the chest or the abdomen (belly). 
• Stage II (2). The lymphoma is not in the chest and is one of these:
  
  • It's only one tumor and is in lymph nodes close to it
  • It's in two or more places, but it’s all in either the upper or lower part of the body (either all above or all below the diaphragm)
  • The lymphoma started in the digestive tract and all of it can be removed with surgery, including any lymph nodes that have cancer in them.
• Stage III (3). Can be any of these:
  
  • The lymphoma is more than 1 tumor outside of the lymph nodes and might be in both the upper and lower parts of the body (it's above and below the diaphragm). It might also be in the bones or skin
  • It started in the chest
  • It started in the abdomen (belly) and is too widespread to be removed with surgery
  • There's lymphoma next to the spine and maybe in other parts of the body
  • There's lymphoma in more than 1 set of lymph nodes above. And it's above and below the diaphragm
  • There's a lymphoma tumor in a bone that has spread to nearby tissues
• Stage IV (4). The lymphoma is in the bone marrow or the central nervous system (the brain and spinal cord) when it's first found. It may also be in other parts of the body.

Treatment will depend on the lymphoma type and stage. Non-Hodgkin lymphoma can be treated with any of these:

• Chemotherapy. Medicines that kill cancer cells or stop them from growing are used for this treatment. They may be given by IV (intravenous) into the vein, injected into tissue (as a shot), or taken by mouth. Chemo is the main treatment for NHL.
• Immunotherapy. These medicines work with your child's immune system to find and kill cancer cells.
• Surgery. Surgery may be done to remove tumors.
• Targeted therapy. These medicines target certain parts of cancer cells to kill them without harming a lot of healthy cells. The NHL cells must be tested to see if they have the targets these medicines work on.
• High-dose chemotherapy with a stem cell transplant. Young blood cells (called stem cells) are taken from the child or from someone else. The child is then given a large amount of chemotherapy to damage or destroy the bone marrow. After the chemo, the stem cells are put into the blood. Over time, they rebuild the bone marrow.
• Radiation therapy. This treatment uses high-energy X-rays or other types of radiation to kill cancer cells or help keep them from growing. It's not often used to treat NHL in children. But it might be used to treat serious problems caused by NHL tumors. For instance, it can shrink a tumor that's causing pressure or pain.
• Supportive care. Every kind of cancer treatment can cause side effects. Medicines and other treatments can be used to ease or help prevent side effects like pain, fever, infection, nausea and vomiting.
• Clinical trials. Ask your child's health care provider if there are any treatments being tested that may work well for your child. Most children with cancer are treated as part of a clinical trial.

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

• Check on their response to the treatment
• Manage the side effects of treatment
• Look for returning or spreading cancer

With treatment, most children with NHL 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 outcomes. 
• Ongoing follow-up care during and after treatment is needed.
• New treatments are being tested to improve outcomes and to reduce side effects.
• Most children with cancer are cured.

Possible complications depend on the type and stage of the lymphoma. They also vary a lot based on where the lymphoma is and how it's treated. Problems can include things like:

• Heart and blood vessel damage
• Lung problems
• Changes in thinking, learning and memory
• Increased chance of other cancers later in life
• Trouble having children (infertility)
• Developmental and growth delays
• Death

Treatment may also cause side effects, such as:

• Increased risk of bleeding
• Increased risk for infection
• Nausea and vomiting
• Diarrhea
• Poor appetite
• Sores in the mouth
• Hair loss

Many treatment side effects can be dealt with to keep them from getting worse. There may even be things you can do to help prevent some of them. Most side effects go away over time after treatment ends. But some treatments can cause long-term side effects that may not show up until many years after your child finishes treatment. Ask your child's treatment team what you might expect.

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

• 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.
• 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.
• If your child uses tobacco, help them quit. If your child doesn’t use tobacco, make sure they know the dangers of it and don't start. Also keep your child away from other people's tobacco smoke.

Talk to your child's treatment team about problems you should watch for. Contact the health care provider if your child has:

• Symptoms that get worse
• New symptoms
• Side effects from treatment

It's important to know what problems to watch for and when you need to call the health care provider. Be sure you know what number to call to get help after office hours and on weekends and holidays.