Non-Hodgkin Lymphoma in Children

We know how difficult a diagnosis of non-Hodgkin lymphoma (NHL) can be for parents and young patients because we have treated hundreds of children with all types of NHL. From your child’s first appointment, throughout their treatment and beyond, CHOC’s Lymphoma Treatment Program is dedicated to provide exceptional care, with compassion and open communication.

What is non-Hodgkin lymphoma?

Non-Hodgkin lymphoma (NHL) is a type of cancer that affects the lymphatic system, a part of the immune system that helps fight diseases and infections. NHL starts when lymphocytes, a type of white blood cell, grow out of control. Over time, this causes tumors to grow in the body’s lymphatic system. The cells can also spread to other organs and tissues in the body. NHL is a rare disease in children. It can occur at any age and affects boys more often than girls. There are different types of NHL in children, many of which are divided into T-cell and B-cell lymphomas.

What are the different types of non-Hodgkin lymphoma in children?

Non-Hodgkin lymphomas (NHL) can be divided into T-cell and B-cell lymphomas, referring to the type of white blood cell where the cancer starts. CHOC treats all types of NHL diagnosed in children, including three of the most common types and sub-sets of:

• Burkitt Lymphoma and Diffuse Large B-Cell Lymphoma
• Lymphoblastic Lymphoma
• Anaplastic Large Cell Lymphoma

CHOC has unique expertise in successful treatment of some rare types of pediatric NHL including:

• Peripheral T-Cell Lymphoma (PTCL)
• NK T-cell Lymphoma (NKTL)
• Hepatosplenic T-Cell Lymphoma
• CNS Lymphoma
• Epstein-Barr Virus-associated Lymphoma

What are the causes and risk factors of non-Hodgkin lymphoma?

The exact cause of non-Hodgkin lymphoma is not known. Researchers believe that genetics, and the presence of some viral infections, like the one that causes mono, may increase a child’s risk for developing the disease.
Risk factors include having:

• Epstein-Barr virus, the virus that causes mono (mononucleosis)
• Certain hereditary immune system conditions
• A weakened immune system due to HIV or other autoimmune disorders

What are the symptoms?

Often children do not experience symptoms of non-Hodgkin lymphoma until the cancer has progressed.
Symptoms may include:

• Swollen or enlarged lymph nodes
• Chills, fever or night sweats not due to an infection
• Unexpected weight loss
• Shortness of breath or cough
• Chest pain or pressure
• Abdominal pain and distention
• Obstruction of blood flow, swollen neck and face
• Metabolic problems, such as kidney failure
• Fatigue and tiredness
• Severe or frequent infections
• Bruising or bleeding easily
• Headaches, blurred or double vision, changes in speech

How is NHL diagnosed and staged in children?

Because of the severity of the symptoms and the aggressive nature of NHL, many cases are diagnosed in the emergency room. Your doctor will ask about your child’s medical history and health history. After examining your child, they may order several tests to diagnose and classify your child’s type of NHL. These can include:

• Blood and urine tests.
• Lymph node biopsy. This type of biopsy is needed to diagnose NHL. A sample of tissue is taken from the lymph nodes (or other areas of concern) and checked for the presence of cancer cells.
• Chest X-ray. This will show if cancer has spread to lymph nodes in the chest.
• CT scan. This scan is used to check the abdomen, chest and pelvis for cancer cells.
• MRI scan. This highly detailed scan checks for cancer cells in the brain and spinal cord. It may be used to get a better look if X-ray or CT scans are unclear.
• Ultrasound. Also called sonography. Sound waves and a computer are used to view lymph nodes and internal organs.
• PET scan. This scan uses a radioactive agent that’s injected into the bloodstream to pinpoint areas of the body that may contain cancer cells. It is often combined with a CT scan and called a PET/CT scan.
• Bone marrow aspiration and biopsy. These tests may be done to see if cancer cells have reached the bone marrow – the soft tissue in the bones that helps make blood cells.
• Lumbar puncture (spinal tap). During this test, a small amount of cerebral spinal fluid (CSF) is removed from the lower back to check the brain and spinal cord for cancer cells.
• Pleural or peritoneal fluid sampling. Fluid is removed from the lining around the lungs (pleura) and abdomen (peritoneum) to look for cancer cells.

Staging

A cancer diagnosis also includes staging, a process that defines how far, and where, the cancer has progressed. Staging is an important part of defining your child’s treatment plan.

• Stage I: The lymphoma is in one location. It’s either in a lymph node or one other part of the body. It did not start in the chest or abdomen.
• Stage II: The lymphoma is in two or more places. It’s all in either the upper or lower part of the body. Or it started in the digestive tract and can be removed by surgery. It’s not in the chest.
• Stage III: The lymphoma is in the chest, or it has spread throughout the abdomen. Or it is both in the upper and lower parts of the body (above and below the diaphragm), but is not in bone marrow, or in the brain or spinal cord (central nervous system).
• Stage IV: The lymphoma is in the bone marrow, the brain or spinal cord, or all three when it is first discovered.

How is NHL treated?

At CHOC, we follow standardized protocols established by the Children’s Oncology Group and personalize them for your child. Depending on their lymphoma type, stage and other health factors, your child may receive treatments such as:

• Chemotherapy. Our approach to chemotherapy is short, targeted and intensive.
• Monoclonal antibodies. This type of therapy targets and kills the cancer cells without harming healthy cells.
• Surgery. In rare occasions, surgery may be performed to remove tumor in the abdomen.
• Stem cell transplant (or bone marrow transplant). First, healthy, young blood cells (stem cells) are taken from the patient or from a donor like a family member. Next, your child will receive high-dose chemotherapy medicine which kills cancer cells, but damages the bone marrow. After chemotherapy, the stored stem cells are put back into the child’s bloodstream to replace the damaged bone marrow.