Non-Hodgkin Lymphoma In Children

What is Lymphoma?

Lymphoma is a type of cancer that develops in the lymphatic system, a critical part of the body’s immune system responsible for fighting infections. It develops when lymphocytes, a type of white blood cell, start growing in an abnormal, uncontrolled way.

Lymphomas are the third most common group of cancers in children aged 0 to 14, after leukemias and other central nervous system (CNS) tumors.

What are the types of lymphoma?

There are many types of lymphoma. It is important to know what type of lymphoma the child has, as each type is treated differently.

The 2 main groups of lymphoma:

Hodgkin lymphoma
Non-Hodgkin lymphoma.

There are several types of Hodgkin lymphoma, but they are all usually treated in the same way. On the other hand, the various types of non-Hodgkin lymphomas need different treatments.

Most of the lymphomas seen in children are high-grade (rapidly growing) lymphomas. Although this may sound alarming, high-grade lymphomas are very likely to go into remission (no evidence of lymphoma) with the right treatment. In general, children have a better outcome than adults and cure rates are improving all the time.

What are the symptoms of lymphomas?

Lymphoma can be difficult for doctors to diagnose. Many of the symptoms (signs of illness) of lymphoma are also seen in other, less serious conditions. Children who have the same type of lymphoma can have different symptoms. The child’s symptoms will depend on where in their body the lymphoma is growing.

Swollen lymph nodes

The first symptom that most people notice is a lump or several lumps that do not go away after several weeks. You might be able to feel one or more lumps in the child’s neck, armpit or groin. These lumps are swollen lymph nodes, where abnormal lymphocytes are growing.

There are other common symptoms of lymphoma. The child might have some of these symptoms or no other symptoms apart from lumps. Everyone’s symptoms are different, but common ones include:

Children with Burkitt lymphoma often develop large masses, usually in their abdomen or bowel. If this happens, the child might have serious abdominal swelling, pain and vomiting, or a build-up of fluid in their abdomen
Fatigue (extreme tiredness)
Drenching sweats, especially at night – you might need to change their nightclothes and bedding
Fevers (high temperature)
Unexplained weight loss
Itching

Night sweats, unexplained weight loss and fevers often occur together. These three symptoms are called ‘B symptoms’. B symptoms can sometimes effect how the child needs to be treated.

How is the lymphoma diagnosed?

When lymphoma is suspected, doctors will order a number of tests.

Blood tests such as complete blood cell, liver and kidney function test, LDH, ESR etc.
Chest X-ray to show the heart, lungs, and other parts of the chest.
Lymph node biopsy by taking a sample of tissue from the lymph nodes. It’s checked with a microscope for cancer cells. A lymph node biopsy is needed to diagnose Hodgkin lymphoma.
CT scan for the abdomen, chest, and/or pelvis. A CT scan uses a series of X-rays and a computer to make detailed pictures of the body.
MRI scan. An MRI uses large magnets, radio waves, and a computer to make detailed pictures 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.
Positron emission tomography (PET) scan. For this test, a radioactive sugar is injected into the bloodstream. Cancer cells use more sugar than normal cells, so the sugar will collect in cancer cells. A special camera is used to see where the radioactive sugar is in the body. A PET scan can sometimes spot cancer cells in different areas of the body, even when they can’t be seen by other tests. This test is often used in combination with a CT scan, in which case it is called a PET/CT scan.
Some children need a bone marrow biopsy and a lumbar puncture. These tests are usually done under general anesthesia

How is lymphoma staged?

Staging is the process of checking if and where the cancer has spread. Staging also helps to decide the treatment. The results of the aforementioned tests help the doctors stage to stage the lymphoma. Staging describes how much lymphoma the child has in their body and where it is. It is important to know the stage of the lymphoma as it helps doctors plan how much treatment the patient needs. There are different staging systems, but across the board the stages of lymphoma range from 1 to 4, with 1 being the earliest and 4 being the most advanced stage. The stage may be written by using Roman numerals: I, II, III or IV. Lymphomas that are stage 1 or 2 are often referred to as ‘localized’; whereas stages 3 and 4 can be called ‘extensive’ or ‘advanced-stage’. This may sound alarming but many children have advanced-stage lymphoma when it is diagnosed, and most children have a very good outlook.

How is lymphoma treated?

Treatment for Hodgkin lymphoma may include:

Chemotherapy: This treatment uses medicines to kill cancer cells and stop their growth. There are lots of different chemotherapy drugs used to treat lymphoma. The best treatment regimens for each type of lymphoma have been worked out over many years in clinical trials, and research continues to improve the treatment. Usually, several drugs that destroy cells in different ways are given together at the same time. Each combination of drugs is known as a drug regimen. Chemotherapy is given in cycles. A cycle is a block of chemotherapy that is followed by some time off treatment to let the child’s body recover. Chemotherapy kills healthy cells as well as lymphoma cells, so the recovery time is needed for healthy cells to build back up. Most children with lymphoma have several cycles of chemotherapy over several months. They might have to stay in the hospital for some of their treatment, whereas some treatment might be given as an outpatient (where the child goes into hospital for a few hours at a time to have the treatment but doesn’t have to stay overnight). The number of cycles of chemotherapy that the child needs depends on the type and stage of the lymphoma. Children with lymphoma are likely to have an evaluation after their first two or three cycles of chemotherapy. The results of the scan are used to decide whether the child needs more chemotherapy, and if he needs radiotherapy after completing their chemotherapy.
Antibody therapy: Everyone’s body makes antibodies naturally to fight infection. Each antibody sticks to a target (a certain protein) on cells such as bacteria, and tells your body to get rid of the cell. Laboratory-made antibodies work in the same way and attach to a specific target to tell the body to destroy it.
Stem cell transplant (bone marrow transplant): This treatment takes a patient's (or a donor's) cells from their bone marrow or blood and transplants them to the patient after chemo.
Radiation therapy: This treatment uses high-energy X-rays to shrink tumors and prevent them from growing. It is also called X-ray therapy. Some children with Hodgkin lymphoma have radiotherapy. Radiotherapy is usually only given if your child still has some lymphoma in their body after their first two cycles of chemotherapy. In this case, it is given after the course of chemotherapy has been completed. Radiotherapy is given over several days or weeks but each treatment only lasts a few minutes. Patients with non-Hodgkin lymphoma usually don’t treated with this method.
Surgery: Surgery is rarely used to treat lymphoma as it is likely to leave behind some lymphoma cells and chemotherapy would still be needed.

Childhood Lymphoma: Outcomes and Prognosis

In general, childhood lymphoma is considered highly “curable”. Hodgkin lymphoma in particular has been found to be very sensitive to both chemotherapy and radiation therapy, and was, in fact, the first form of cancer to be “cured” with radiation therapy alone or with a combination chemotherapy protocol. As with all childhood cancers however, the five-year survival rate for Hodgkin and the one-year survival for non-Hodgkin depend upon the unique nature of each child’s specific disease, the stage of the disease upon diagnosis, and the categorization of risk. For children and adolescents with Stage I or Stage II lymphoma, the overall survival rate is over 90-95%; however, for children and adolescents with Stage III or Stage IV Hodgkin lymphoma, the overall survival rate may be as low as 80%.