All About Pediatric Acute Leukemia
What is leukemia?
Leukemia is a cancer of the blood or blood cells. Leukemia is classified by:
- How quickly the leukemia develops. It can be acute (developing quickly) or chronic (developing more slowly over time).
- The type of white blood cells that are affected (either lymphoid or myeloid).
Pediatric acute leukemia is leukemia that develops quickly in children aged 0 to 19 years. Read about the types of leukemia to learn more.
This article will focus on pediatric acute leukemias. These include acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML).
What causes leukemia and who is at risk?
Leukemia is the most common pediatric cancer. The exact cause of pediatric leukemia is not known, and it continues to be studied. There are a few known risk factors, such as:
- Genetic syndromes including Down syndrome, Li-Fraumeni syndrome, neurofibromatosis, and Fanconi anemia.
- Having a poorly functioning immune system, either inherited or caused by a medicine or disease. Some children are born with (inherited) immune system problems, including those with Ataxia-telangiectasia, Wiskott-Aldrich syndrome, Bloom syndrome, and Shwachman-Daimond syndrome.
- Having a sibling with leukemia.
- Exposure to radiation, chemotherapy, and certain chemicals.
What are the signs and symptoms?
Many of the symptoms of leukemia are nonspecific, meaning symptoms (like fatigue, weight loss, aches and pains) could be caused by other health problems. Leukemia affects the blood, so many of the symptoms are caused by abnormal numbers of blood cells in your body.
- Not enough red blood cells can make you feel tired, weak, cold, dizzy, short of breath, and look pale.
- A low platelet count can cause you to have bleeding and bruising more easily.
- A low or very high white blood cell count can cause fevers and infections.
Some other symptoms are:
- Bone and joint pain. This is caused by the bone marrow being full of immature blood cells.
- Stomach pain, loss of appetite, and weight loss. Leukemia cells can build up in the organs in your belly, like your kidneys, liver, and spleen. This can cause the organs to grow bigger than normal. This pain and discomfort can cause appetite and weight loss.
- Swollen lymph nodes. Your lymph nodes work to filter and clean your blood. Leukemia cells can build up in the lymph nodes and can make them swell or get bigger than normal.
There are other symptoms of leukemia depending on how the cancer is affecting your body. The important thing is to check in with your child's provider if they are not feeling well.
How is pediatric leukemia diagnosed?
If a provider thinks that your child may have leukemia, your child will first have a full physical exam. The provider may ask the child about how they are feeling and what symptoms they have been having. The provider may order other tests, such as:
- Bone marrow biopsy: A piece of tissue is removed to check for cancer cells. A small amount of fluid or tissue is taken from the bone marrow. A needle is placed into a bone, often the hip bone, to test the fluid or tissue. The process can be painful, but every effort will be made to keep a child comfortable for the test.
- Blood tests. A complete blood count (CBC) will be done to count and look at the different types of blood cells. Tests will check how well the kidneys and liver are working. Your provider may also test for genetic changes or abnormalities.
- A child may also need a CT scan, MRI, X-ray, and ultrasound to look at certain parts of their body.
- Lymph node biopsy: In some cases, a lymph node may be removed and looked at for cancer cells.
- Lumbar Puncture: If there is concern that there are cancer cells in the cerebrospinal (CSF) fluid, a lumbar puncture may be done. The cerebrospinal fluid is the fluid that surrounds the brain and spinal cord. A needle is placed in the lower back and into the spinal canal to remove some fluid for testing. The pressure in the brain can also be checked during this procedure. Providers will make sure that your child is comfortable for the procedure.
The results of these tests will help the care team come up with the best treatment plan for your child.
How is pediatric acute leukemia treated?
Treatment for children with acute leukemia is often split into phases:
- Phase 1: Induction chemotherapy (at the time of diagnosis).
- Phase 2: Post-induction therapy.
- Consolidation/intensification therapy.
- Maintenance therapy.
Phase 1: Remission Induction Chemotherapy
The goal of induction chemotherapy is to stop cancer cells from being made in the bone marrow. “Remission” happens when cancer cells are no longer being made, and the cancer cannot be found. This stage of treatment usually lasts about a month.
Chemotherapy will be given systemically, meaning throughout their whole body, as well as into their central nervous system (CNS). Medication reaches the CNS with intrathecal administration. This treatment is given during a lumbar puncture (LP).
Induction chemotherapy often uses some of the following medications:
- Vincristine.
- Corticosteroid (either prednisone or dexamethasone).
- Asparaginase.
- Intrathecal chemotherapy.
These medications may be used with or without another type of medication called an anthracycline (either doxorubicin or daunorubicin).
Some types of ALL may also be treated with targeted therapy medications, such as imatinib, dasatinib, nilotinib, gemtuzumab ozogamicin, all-trans-retinoic acid, and arsenic trioxide.
Phase 2: Post-Induction Therapy
The goal of post-induction therapy is to help your child stay in remission with more treatment.
- Consolidation/Intensification: Consolidation therapy starts when your child is in remission from induction therapy. The medications, intensity, and dosing depend on your child’s overall health, diagnosis, and risk factors. They will often be on consolidation treatment for a few months. Some medications that may be used during consolidation/intensification are:
Another treatment option during the consolidation phase is a stem cell transplant (also called a bone marrow transplant). Very high doses of chemotherapy are given to kill off the body’s own blood cells. Whole-body radiation may also be given. The body is then "rescued" with new stem cells that will begin to make new blood cells. The transplanted stem cells can either come from blood, bone marrow, or a baby's umbilical cord blood. Your child’s immune system will be very weak during this time. There are serious side effects that can happen, so you will have frequent appointments at the cancer center. There are two different types of stem cell transplants. An allogeneic stem cell transplant uses cells from a donor. The donor's tissue type is tested to make sure it is a match for the patient. An autologous transplant uses the patient's own stem cells.
- Maintenance Therapy: The goal of maintenance treatment is to keep the bone marrow free of cancer cells. Treatment can continue for months to up to 2 years, but the dose is often not as intense. During this stage, a child is monitored for relapse (return of cancer). Some medications that may be used are:
- 6-mercaptopurine (6-MP).
- Methotrexate.
- Vincristine.
- A steroid (prednisone or dexamethasone).
Depending on how your child responds to treatment, they may also receive intrathecal chemotherapy during this phase.
Clinical Trials
There are clinical research trials for most types of cancer, and every stage of the disease. Clinical trials are designed to determine the value of specific treatments. Trials are often designed to treat a certain stage of cancer, either as the first form of treatment offered, or as an option for treatment after other treatments have failed to work. They can be used to evaluate medications or treatments to prevent cancer, detect it earlier, or help manage side effects. Clinical trials are extremely important in furthering our knowledge of disease. It is through clinical trials that we know what we do today, and many exciting new therapies are currently being tested. Talk to your provider about participating in clinical trials in your area. You can also explore currently open clinical trials using the OncoLink Clinical Trials Matching Service.
Follow-up Care and Survivorship
Your child, their caregivers, and providers will work together to talk about the best treatment options for your child. Be sure to ask your provider about potential late or long-term side effects (side effects that can happen after treatment has ended). Talk with your provider about making a survivorship care plan or develop one using the Smart ALACC tool. Your child may benefit from being seen in a survivorship clinic. At a survivorship clinic, a provider can review your child's history and provide recommendations. You can contact cancer centers in your area to see if they have a survivorship clinic or search for a clinic on OncoLink's survivorship clinic list.
Resources for More Information
Blood Cancer United: A global leader in the fight against cancer. Their mission is to cure leukemia, lymphoma, Hodgkin's disease, myeloma, and improve the quality of life of patients and their families.
Childhood Leukemia Foundation: Provides patient education, advocacy, self-esteem, and smiles. All programs are free of charge.
Alex's Lemonade Stand: Their mission is to change the lives of children with cancer through funding impactful research, raising awareness, supporting families, and empowering everyone to help cure childhood cancer.