Childhood Non-Hodgkin's Lymphoma

What is non-Hodgkin’s lymphoma in children?

Non-Hodgkin lymphoma in children is a type of cancer that starts in children’s lymphatic systems. It most commonly affects children ages 5 to 19, although newborn babies may have this condition. Healthcare providers have effective treatments that may cure most children who have non-Hodgkin lymphoma. Some children may develop serious medical conditions or health issues years after completing treatment. These conditions or issues are late effects. Researchers are evaluating new treatments that might cure children without risk of serious late effects.

How does non-Hodgkin’s lymphoma affect my child?

This condition may appear in the lymphoid tissue in any part of children’s bodies, including their central nervous systems. (Lymphoid tissue is part of people’s immune systems, helping to protect them from infections.) Non-Hodgkin lymphoma may start in B-lymphocytes (B-cells), T- lymphocytes (- cells) or natural killer (NK) cells. These cells provide protection from various bacterial and viral infections. There are three major types of childhood non-Hodgkin lymphoma; each type may affect children in different ways.

Mature B-cell lymphomas

These lymphomas include conditions arising from B-cells, and represent more than half of all non-Hodgkin lymphomas in children. More than 90% of children with this type of non-Hodgkin lymphoma condition are cured after treatment. The different B-cell lymphomas include:

• Burkitt lymphoma/leukemia: Burkitt lymphoma and Burkitt leukemia are different forms of the same disease and are treated in very similar ways. This type of non-Hodgkin lymphoma usually starts in lymph nodes located in your child’s belly (abdomen) and chest. But it may also affect parts of your child’s bone marrow, long bones (arm and leg bones) or their central nervous system. In Burkitt leukemia, children have large numbers of abnormal cells in their blood that come from cells in their bone marrow. Both Burkitt lymphoma and leukemia grow very quickly.
• Diffuse large B-cell lymphoma: This form of lymphoma frequently affects more teenagers — children age 12 to 19 — than younger children.
• Primary mediastinal B-cell lymphoma: This type of lymphoma starts in B-cells in the mediastinum, the area behind children’s breastbones. The lymphoma may spread to children’s lymph nodes. This lymphoma type happens more often in older teenagers than in younger children

Lymphoblastic lymphoma

Lymphoblastic lymphoma represents about one-third of all non-Hodgkin lymphoma in children. It’s more common in teenagers than in younger children. Lymphoblastic lymphoma affects B-cells or T-cells.

It usually starts in lymph nodes in children’s mediastinum (the area behind their breastbones) or in children’s necks, but it may also involve lymph nodes in their bellies (abdomen), bone marrow or some organs. More than 80% of children treated for lymphoblastic lymphoma survive their diagnosis.

Anaplastic large cell lymphoma (ALCL)

Anaplastic large cell lymphoma represents about 15% of all childhood non-Hodgkin lymphomas. It affects T-cells, typically starts in children’s lymph nodes, and may affect children’s skin and bones. Approximately 70% of children with ALCL survive their diagnosis.

How common is non-Hodgkin’s lymphoma in children?

Each year, between 800 and 1,000 children are diagnosed with non-Hodgkin lymphoma. It’s one of the most common cancers affecting children. The incidence of non-Hodgkin lymphoma varies depending on children’s ages, and a child’s risk of developing non-Hodgkin lymphoma increases as they grow up.

For example, non-Hodgkin lymphoma affects 6 children per 1 million children age 5 and younger. It affects 10 children and teens per 1 million children and teens, and 15 teens ages 15-19 per every 1 million teens. Children assigned male at birth (AMAB) may be affected more frequently than children assigned female at birth (AFAB), and more white children are diagnosed with this condition than Black children.

What are the symptoms of non-Hodgkin’s lymphoma in children?

Non-Hodgkin lymphoma symptoms may resemble many less serious illnesses. You should contact your child’s healthcare provider if your child has swollen lymph nodes and persistent breathing problems, including chronic cough or wheezing. Other non-Hodgkin lymphoma symptoms include:

• Frequent fevers for no known reason.
• Coughing.
• Night sweats.
• Weight loss for no known reason.
• Swelling of the head, neck, upper body, or arms.
• Trouble swallowing (dysphagia).

What causes non-Hodgkin’s lymphoma in children?

There’s no clear cause for non-Hodgkin lymphoma in children but healthcare providers have identified a few risk factors that may increase children’s risk of developing the condition. It’s important to remember risk factors don’t predict that a child will absolutely develop this condition. If your child has any of the following health issues, ask their healthcare provider about your child’s risk of developing non-Hodgkin lymphoma:

• Your child received treatment for another type of cancer.
• Your child has mononucleosis (Epstein-Barr virus) or human immunodeficiency virus (HIV).
• Your child’s immune system is weak because they had an organ or bone marrow transplant.
• Your child’s immune system is weak because they’re taking medication to suppress their immune system.
• Your child inherited certain diseases that affect their immune system.

Inherited immune system diseases

Inherited diseases that may increase your child’s risk for non-Hodgkin lymphoma include:

• Ataxia-telangiectasia: This is a neurological disease that affects children’s central nervous systems, immune systems and other body systems.
• Chediak-Higashi syndrome: This disorder affects children’s immune systems and nervous systems.
• Common variable immunodeficiency: Children with this condition have low levels of antibodies (proteins that fight infections) in their blood.
• Nijmegen breakage syndrome: Children with this condition have immunodeficiency and low levels of immune system proteins and T-cells.
• Severe combined immunodeficiency disorder (SCID): This disorder affects children’s T-cells and B-cells, weakening their immune systems and creating a high risk for developing certain cancers, including non-Hodgkin lymphoma.
• Wiskott-Aldrich syndrome: This condition affects children’s blood cells and immune system cells. Children who have Wiskott-Aldrich syndrome have fewer platelets than normal. Platelets are blood cells that help your blood clot. Children who have low platelet levels may bruise or bleed more easily. This syndrome also affects the immune system’s T-cells and B-cells.
• X-linked lymphoproliferative syndrome: This disorder affects children’s immune systems. Children with this syndrome may have an abnormally severe response if they’re infected with Epstein-Barr virus.

How do healthcare providers diagnose non-Hodgkin lymphoma in children?

Diagnosing non-Hodgkin lymphoma in children is a painstaking process that typically requires a series of blood and imaging tests and biopsies. Healthcare providers will examine your child for signs of diseases such as lumps, swollen glands or anything that seems unusual. They may obtain detailed information about the health of immediate and remote family members, including any history of cancers.

Blood tests

• Complete blood count (CBC): This test measures your child’s blood cell levels. Healthcare providers may use CBC results to look for signs of non-Hodgkin lymphoma in your child’s bone marrow or to see if treatment affects your child’s bone marrow.
• Blood chemistry studies: Healthcare providers take blood samples to measure the amounts of certain substances released into your child’s blood by their organs or tissues. Unusually high or low amounts of a substance may be a sign of disease.
• Liver function tests: This is another blood test. Healthcare providers measure the amounts of certain substances released into your child’s blood by the liver. A higher-than-normal amount of a substance can be a sign of cancer.

Healthcare providers may also use blood tests to look for viruses linked to non-Hodgkin lymphoma in children.

Imaging tests

• Computed tomography (CT) scan: CT scans make detailed pictures of areas inside your child’s body. Your child’s healthcare provider may do this test to look for enlarged lymph nodes or other signs of lymphoma.
• Magnetic resonance imaging (MRI): This test uses a magnet, radio waves and a computer to make a series of detailed pictures of areas inside your child’s body. Healthcare providers may use MRIs to look for signs of non-Hodgkin’s lymphoma in your child’s brain or spinal cord.
• Positron emission tomography (PET) scan: Healthcare providers use this test to look for lymphoma in your child’s body and/or see how treatment affects their lymphoma. In PET scans, providers inject a very small amount of radioactive glucose (sugar) into one of your child’s veins. (Tracking glucose is one way to check for cancer because cancer cells are more active than normal cells, which means they use up more glucose.) PET scans pinpoint spots in your child’s body where glucose is used.
• Ultrasound: This test uses high-energy sound waves (ultrasound) that bounce off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. Healthcare providers use sonograms to look for lymph nodes near the surface of your child’s body or in their belly, liver and spleen.

Biopsies

Healthcare providers do biopsies to get lymph node, tumor and bone marrow samples they can examine under a microscope or through other lab tests. Biopsies are the only way healthcare providers can confirm their diagnoses. More importantly, biopsy results help them identify specific non-Hodgkin lymphoma types so they can use the treatment that works best to cure the condition.

There are several biopsy types. Providers use the biopsy type that’ll enable them to get enough cells and tissues for examination while causing the fewest side effects. Healthcare providers will give your child sedatives, numbing medication, or local or general anesthesia. That way, your child isn’t uncomfortable during the procedure. Providers may use:

• Surgical biopsy: Healthcare providers may do surgery to remove an entire lymph node, lump of tissue or a small part of a large tumor.
• Needle biopsies: Healthcare providers do core biopsies or fine-needle biopsies to remove tissue or parts of your child’s lymph node.

Other biopsy types

If your child has non-Hodgkin lymphoma, their healthcare provider may do other tests to collect tissue so they can look for signs lymphoma has spread. Just like initial biopsies, your child will receive sedatives and/or anesthesia so they aren’t uncomfortable. Those biopsies include:

• Lumbar puncture (spinal tap): Healthcare providers may do lumbar punctures to see if your child has cancer that’s spread to their brain and spinal cord.
• Bone marrow aspiration and bone marrow biopsy: Healthcare providers do these tests to see if lymphoma has spread to your child’s bone marrow. To do this test, providers insert a hollow needle into your child’s hipbone so they can remove bone marrow and a small sample of bone.
• Thoracentesis: Lymphoma may spread to the membrane lining the inside of your child’s chest or lung, causing fluid to build up. Healthcare providers use a hollow needle to draw out fluid so they can examine the fluid for signs of cancer cells.
• Paracentesis: This test pulls fluid from inside your child’s belly (abdomen).
• Mediastinoscopy: This is surgery done under general anesthesia. Healthcare providers do this test to look for abnormal areas in your child’s organs, tissues and lymph nodes between your child’s lungs. They do this test with a mediastinoscope. This is a thin tube-like instrument that has a light and lens for viewing and a tool to remove tissue or lymph samples. They insert the mediastinoscope through a cut at the top of your child’s breastbone.

Do healthcare providers stage non-Hodgkin’s lymphoma in children?

Yes, healthcare providers do have stages or classifications for non-Hodgkin lymphoma. They use cancer staging systems to develop treatment plans.

Non-Hodgkin lymphoma stages

Stage I: Healthcare providers find cancer in one area either above or below your child’s diaphragm, the large muscle that separates your child’s chest and belly.

Stage II: They find cancer in one area and in surrounding lymph nodes, in two or more lymph nodes or on other areas on the same side of your child’s diaphragm.

Stage III: They find cancer in any of the four situations below:

• There’s cancer in tissue or lymph nodes on both sides of your child’s diaphragm.
• There’s cancer in your child’s chest.
• There’s cancer in your child’s belly that providers can’t remove with surgery.

Stage IV: They find cancer in your child’s bone marrow, spinal cord and/or their brain.

How do healthcare providers treat non-Hodgkin lymphoma in children?

Healthcare providers constantly evaluate new treatments that can treat lymphoma without causing significant late effects. Late effects are serious medical conditions or issues that may occur years after the treatment was completed and children don’t have non-Hodgkin lymphoma. The six main treatment types are:

• Chemotherapy: Chemotherapy is the primary treatment for children who have non-Hodgkin lymphoma. Healthcare providers may use one medication at a time or combine medications.
• Surgery: In some instances, surgeons may remove tumors during biopsies. In these instances, children would still need additional treatment such as chemotherapy or immunotherapy.
• Targeted therapy: Targeted therapy affects specific cancer genes, proteins or small molecules inside the lymphoma cells that help the lymphoma cells survive the effect of chemotherapy.
• Immunotherapy: This treatment works by stimulating your child’s immune system, or by using artificial components of the immune system to destroy lymphoma cells.
• Radiation therapy: In some instances, healthcare providers may use radiation therapy to kill any remaining cancer cells.
• Stem cell transplantation: Healthcare providers may use stem cells taken from blood or bone marrow to replace unhealthy cells with healthy cells.

What are cancer treatment late effects?

Cancer treatment late effects are medical conditions or issues that can develop months or years after people complete treatment. Some late effects may be life-threatening. As more children live longer after treatment for non-Hodgkin lymphoma, healthcare providers are working to find and treat late effects as early as possible. Common late effects in children with non-Hodgkin lymphoma include:

• Secondary cancers: As its name implies, secondary cancer happens when children get a new type of cancer after non-Hodgkin lymphoma treatment. Common secondary cancers include acute myeloid leukemia, myelodysplastic syndrome, breast cancer and thyroid cancer.
• Heart conditions: Some children who received chemotherapy may develop heart problems, including congestive heart failure, weakened heart (cardiomyopathy), hardening or narrowing of the arteries (coronary artery disease), abnormal heartbeat (arrhythmia) or scarring of their hearts’ protective lining (pericarditis.)
• Pneumonitis (inflammation of the lungs): Certain chemotherapy drugs may affect children’s lungs.
• Liver problems: Some children who receive certain chemotherapy drugs may develop enlarged livers or cirrhosis of the liver.

What about clinical trials for non-Hodgkin’s lymphoma in children?

Your child’s healthcare provider may recommend that your child participate in clinical trials for non-Hodgkin lymphoma treatment. In a clinical trial, people receive standard of care or they receive treatments under evaluation.

Standard of care treatments are approved medications and other treatments that have proven to provide the best results. Trial treatments, as the name suggests, are treatments offered as part of a trial to see if the treatments improve on standard of care treatments or if the trial treatments have the same result but cause fewer late effects.

Clinical trials may involve new drugs, new doses of existing drugs or new combinations of existing treatments. Most of the time, providers at pediatric cancer centers oversee these sorts of clinical trials. Ask your child’s healthcare provider if a clinical trial may be a treatment option for your child.

How can I reduce the chance my child will have non-Hodgkin lymphoma?

Children who have weakened immune systems may be more likely to develop non-Hodgkin lymphoma. For example, if your child needed a bone marrow or organ transplant, they may be at risk for non-Hodgkin lymphoma. Likewise, your child may have increased risk for non-Hodgkin lymphoma if they inherited certain diseases that affect their immune system.

Your child’s healthcare provider knows your child’s health history. If you’re concerned, ask your child’s provider if your child has any conditions that may put them at risk of developing the condition.

That way, you’ll know if you need to watch for specific symptoms that may be early signs of non-Hodgkin lymphoma.

Is non-Hodgkin’s lymphoma curable in children?

Yes, healthcare providers have treatments that can cure most children who have non-Hodgkin lymphoma.

What is the survival rate of non-Hodgkin lymphoma in children?

The survival rate for children who have non-Hodgkin lymphoma varies depending on the lymphoma type. Between 70% and 90% of children are alive for more than five years after diagnosis.

What happens after my child completes treatment for non-Hodgkin’s lymphoma?

You and your child are probably relieved and elated to be through with cancer treatment. Take a minute to celebrate, but remember your child will need to keep in touch with their healthcare provider for a few years to come.

Your child’s healthcare provider may recommend regular follow-up examinations and tests for the first few years post-treatment. That way, they can keep track of your child’s overall health, watch for signs cancer has come back and monitor any late effect symptoms. Your child may need blood tests and imaging tests.

How do I/does my child manage cancer treatment late side effects?

Children can have cancer treatment late effects, or late side effects, months and years after they finish treatment. Knowing that, you and your child may feel anxious about the future. It may help to know your child’s healthcare provider will monitor your child’s post-treatment overall health so they can identify and treat any problems early on.

Ask your child’s healthcare provider what, if any, late effects your child may encounter, including specific symptoms that may be early signs of trouble. As your child becomes a young adult who’s managing their health on their own, encourage them to be vigilant about late effect symptoms.

My child has non-Hodgkin lymphoma. How can I help them?

Cancer is hard on everyone, but especially hard on children. Young children may not be old enough to understand why they don’t feel well. They may be apprehensive about tests and treatments. Teens who understand their illness may be angry about their situation and understandably afraid. If you and your child are struggling with cancer’s impact, ask their healthcare provider about child life services. Child life service providers specialize in helping children and their families cope with cancer’s challenges.

A note from Cleveland Clinic

One moment you have a healthy child who has what you think is an everyday illness. The next, you have a child with cancer. It can’t be easy to learn your child has non-Hodgkin lymphoma. Even though healthcare providers cure most children who have this condition, your child still faces intensive medical treatment that will disrupt their daily life. Even after treatment, your child may develop late effects, serious medical conditions or issues that require additional treatment. That’s a lot to absorb. You may feel like your world has turned upside down and wonder if it’ll ever be right side up again.

Your child’s healthcare provider understands what you and your child are going through. Ask them for help, whether it’s information about your child’s situation or resources for you and your family. They’re happy to do everything they can.