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Childhood Acute Lymphoblastic Leukemia Treatment (PDQ®)
Topics in This Section
- General Information About Childhood Acute Lymphoblastic Leukemia
- Stages of Childhood Acute Lymphoblastic Leukemia
- Recurrent Childhood Acute Lymphoblastic Leukemia
- Treatment Option Overview
- Treatment Options for Childhood Acute Lymphoblastic Leukemia
- Untreated Childhood Acute Lymphoblastic Leukemia
- Childhood Acute Lymphoblastic Leukemia Subgroups
- Recurrent Childhood Acute Lymphoblastic Leukemia
- Get More Information From NCI
- Changes to This Summary (02/14/2008)
- About PDQ
Childhood acute lymphoblastic leukemia (also called acute lymphocytic leukemia or ALL) is a cancer of the blood and bone marrow. This type of cancer usually gets worse quickly if it is not treated. It is the most common type of cancer in children.
Normally, the bone marrow makes blood stem cells (immature cells) that develop into mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell..
The myeloid stem cell develops into one of three types of mature blood cells:
- Red blood cells that carry oxygen and other materials to all tissues of the body.
- Platelets that help prevent bleeding by causing blood clots to form.
- Granulocytes (white blood cells) that fight infection and disease.
The lymphoid stem cell develops into a lymphoblast cell and then into one of three types of lymphocytes (white blood cells):
- B lymphocytes that make antibodies to help fight infection.
- T lymphocytes that help B lymphocytes make the antibodies that help fight infection.
- Natural killer cells that attack cancer cells and viruses.
In ALL, too many stem cells develop into lymphoblasts or lymphocytes. These cells may also be called leukemic cells. The leukemic cells are not able to fight infection very well. Also, as the number of lymphocytes increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may lead to infection, anemia, and easy bleeding.
This summary is about acute lymphoblastic leukemia. See the following PDQ summaries for information on other types of leukemia:
- Adult Acute Lymphoblastic Leukemia Treatment.
- Chronic Lymphocytic Leukemia Treatment.
- Adult Acute Myeloid Leukemia Treatment.
- Childhood Acute Myeloid Leukemia/Other Myeloid Malignancies Treatment.
- Chronic Myelogenous Leukemia Treatment.
- Hairy Cell Leukemia Treatment.
Four of the subgroups of ALL are based on the type of blood cell that is affected, whether there are certain changes in the chromosomes, and age at diagnosis:
- T cell ALL.
- Philadelphia chromosome positive ALL.
- ALL diagnosed in an infant.
- ALL diagnosed in children who are aged 10 and older and adolescents (teenagers).
These subgroups are treated differently from other types of ALL. (See the Childhood Acute Lymphoblastic Leukemia Subgroups section for more information.)
Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. Possible risk factors for ALL include the following:
- Having a brother or sister with leukemia.
- Being white or Hispanic.
- Living in the United States.
- Being exposed to x-rays before birth.
- Being exposed to radiation.
- Past treatment with chemotherapy or other drugs that weaken the immune system.
- Having certain genetic disorders, such as Down syndrome.
These and other symptoms may be caused by childhood ALL. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:
- Fever.
- Easy bruising or bleeding.
- Petechiae (flat, pinpoint spots under the skin caused by bleeding).
- Bone or joint pain.
- Painless lumps in the neck, underarm, stomach, or groin.
- Pain or feeling of fullness below the ribs.
- Weakness or feeling tired.
- Loss of appetite.
The following tests and procedures may be used:
- Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
- Complete blood count (CBC) with differential: A procedure in which a sample of blood is drawn and checked for the following:
- The number of red blood cells and platelets.
- The number and type of white blood cells.
- The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
- The portion of the sample made up of red blood cells.
Complete blood count (CBC). Blood is collected by inserting a needle into a vein and allowing the blood to flow into a tube. The blood sample is sent to the laboratory and the red blood cells, white blood cells, and platelets are counted. The CBC is used to test for, diagnose, and monitor many different conditions.
- Bone marrow aspiration and biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer.
- Cytogenetic analysis: A laboratory test in which the cells in a sample of blood or bone marrow are viewed under a microscope to look for certain changes in the chromosomes in the lymphocytes. For example, in ALL, part of one chromosome is moved to another chromosome. This is called the “Philadelphia chromosome.”
Philadelphia chromosome. A piece of chromosome 9 and a piece of chromosome 22 break off and trade places. The bcr-abl gene is formed on chromosome 22 where the piece of chromosome 9 attaches. The changed chromosome 22 is called the Philadelphia chromosome.
- Immunophenotyping: A test in which the cells in a sample of blood or bone marrow are looked at under a microscope to find out if malignant lymphocytes (cancer) began from the B lymphocytes or the T lymphocytes.
- Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.
- Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
The prognosis (chance of recovery) and treatment options depend on:
- Age and white blood cell count at diagnosis.
- How quickly and how low the leukemia cell count drops after initial treatment.
- Gender and race.
- Whether the leukemia cells began from the B lymphocytes or the T lymphocytes.
- Whether there are certain changes in the chromosomes of lymphocytes.
- Whether the leukemia has spread to the brain and spinal cord.
- Whether the child has Down syndrome.
The extent or spread of cancer is usually described as stages. For childhood acute lymphoblastic leukemia (ALL), risk groups are used instead of stages. The following tests and procedures may be used to determine the risk group:
- Lumbar puncture: A procedure used to collect cerebrospinal fluid from the spinal column. This is done by placing a needle into the spinal column. This procedure is also called an LP or spinal tap.
- Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
- Testicular biopsy: The removal of cells or tissues from the testicles so they can be viewed under a microscope by a pathologist to check for signs of cancer. This procedure is done only if there seems to be anything unusual about the testicles during the physical exam.
Risk groups are described as:
- Standard (low) risk: Includes children aged 1 to 9 years who have a white blood cell count of less than 50,000/µL at diagnosis.
- High risk: Includes children younger than 1 year or older than 9 years and children who have a white blood cell count of 50,000/µL or more at diagnosis.
It is important to know the risk group in order to plan treatment.
Recurrent childhood ALL is cancer that has recurred (come back) after it has been treated. The leukemia may come back in the blood and bone marrow, brain, testicles, spinal cord, or in other parts of the body.
Different types of treatment are available for children with acute lymphoblastic leukemia (ALL). Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.
Because cancer in children is rare, taking part in a clinical trial should be considered. Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.
Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health professionals who are experts in treating children with leukemia and who specialize in certain areas of medicine. These may include the following specialists:
- Hematologist.
- Medical oncologist.
- Pediatric surgeon.
- Radiation oncologist.
- Endocrinologist.
- Neurologist.
- Neuropathologist.
- Neuroradiologist.
- Pediatric nurse specialist.
- Social worker.
- Rehabilitation specialist.
- Psychologist.
Regular follow-up exams are very important. Side effects can result from treatment long after it ends. These are called late effects. Radiation therapy to the head may affect the child's developing brain and cause changes in mood, feelings, thinking, learning, or memory. Late effects of treatment for ALL also include the risk of second cancers (new types of cancer), especially brain tumors. Early diagnosis and treatment of these secondary brain tumors may help lower the risk from these brain tumors. Children younger than 4 years have a higher risk of side effects from radiation therapy to the brain. It is important to talk with your child's doctors about the possible late effects caused by some treatments. See the PDQ summary on Late Effects of Treatment for Childhood Cancer.
The treatment of childhood ALL is done in phases:
- Induction therapy: This is the first phase of treatment. Its purpose is to kill the leukemia cells in the blood and bone marrow. This puts the leukemia into remission. This is also called the remission induction phase.
- Consolidation/intensification therapy: This is the second phase of therapy. It begins once the leukemia is in remission. The purpose of consolidation/intensification therapy is to kill any remaining leukemia cells that may not be active but could begin to regrow and cause a relapse.
- Maintenance therapy: This is the third phase of treatment. Its purpose is to kill any remaining leukemia cells that may regrow and cause a relapse. Often the cancer treatments are given in lower doses than those used for induction and consolidation/intensification therapy. This is also called the continuation therapy phase.
Bone marrow biopsy and aspirates are done throughout all phases to see how well the leukemia is responding to treatment.
Treatment called central nervous system (CNS) sanctuary therapy is usually given during each phase of therapy. Because chemotherapy that is given by mouth or injected into a vein may not reach leukemia cells in the CNS (brain and spinal cord), the cells are able to find “sanctuary” (hide) in the CNS. Intrathecal chemotherapy and radiation therapy are able to reach leukemia cells in the CNS and are given to kill the leukemia cells and prevent the cancer from recurring (coming back). CNS sanctuary therapy is also called CNS prophylaxis.
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column (intrathecal), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type of the cancer being treated.
Intrathecal chemotherapy may be used to treat childhood ALL that has spread, or may spread, to the brain and spinal cord. When used to prevent cancer from spreading to the brain and spinal cord, it is called central nervous system (CNS) sanctuary therapy or CNS prophylaxis. Intrathecal chemotherapy is given in addition to chemotherapy by mouth or vein.
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. External radiation therapy may be used to treat childhood ALL that has spread, or may spread, to the brain and spinal cord. When used this way, it is called central nervous system (CNS) sanctuary therapy or CNS prophylaxis.
Because radiation therapy to the brain can affect growth and brain development in young children, clinical trials are studying new ways of using radiation therapy that may have fewer side effects, including lower doses and fractionation (dividing the total dose of radiation therapy into several smaller, equal doses delivered over a period of several days).
Stem cell transplant is a method of giving chemotherapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. A stem cell transplant using stem cells from a donor who is not related to the patient is being studied in clinical trials.
High-dose chemotherapy is giving high doses of anticancer drugs to kill cancer cells. This treatment often causes the bone marrow to stop making blood cells and can cause other serious side effects. High-dose chemotherapy is usually followed by stem cell transplant to restore the bone marrow. Clinical trials are studying high-dose chemotherapy for certain patients, including children whose ALL does not go into remission after induction therapy.
Imatinib mesylate (Gleevec) is a type of anticancer drug called a tyrosine kinase inhibitor. It blocks the enzyme, tyrosine kinase, that causes stem cells to develop into more white blood cells (granulocytes or blasts) than the body needs.
This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.
Standard treatment of childhood acute lymphoblastic leukemia (ALL) during the induction, consolidation/intensification, and maintenance phases may include the following:
- Combination chemotherapy.
- CNS sanctuary therapy with intrathecal chemotherapy and/or radiation therapy to the brain.
Some of the treatments being studied in clinical trials for childhood ALL include the following:
- Combination chemotherapy with or without intrathecal chemotherapy. Radiation therapy to brain may also be given.
- Combination chemotherapy followed by stem cell transplant using stem cells donated by a brother or sister.
Information about these and other clinical trials is available from the NCI Web site.
Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with untreated childhood acute lymphoblastic leukemia.
Standard treatment of T-cell childhood acute lymphoblastic leukemia (ALL) is usually combination chemotherapy. CNS sanctuary therapy with intrathecal chemotherapy and/or radiation therapy to the brain may also be given. One of the treatments being studied in clinical trials for T-cell childhood ALL is a new kind of anticancer drug.
Treatment of infants with ALL is usually a clinical trial of systemic chemotherapy with intrathecal chemotherapy or chemotherapy followed by a donor stem cell transplant.
Treatment of ALL in older children and adolescents usually involves more aggressive therapy (stronger treatments and higher doses) than that given to children aged 1-9 years.
Standard treatment of Philadelphia chromosome-positive childhood ALL is usually a stem cell transplant using stem cells donated by a brother or sister. One of the treatments being studied in clinical trials for Philadelphia chromosome-positive childhood ALL is imatinib mesylate (Gleevec).
This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.
Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with T-cell childhood acute lymphoblastic leukemia and Philadelphia chromosome positive childhood precursor acute lymphoblastic leukemia.
Standard treatment of recurrent childhood acute lymphoblastic leukemia (ALL) may include the following:
- Combination chemotherapy.
- Systemic chemotherapy and intrathecal chemotherapy with or without radiation therapy to the brain and spinal cord.
- Chemotherapy with stem cell transplant, using stem cells from a donor who is related to the patient, with or without total-body irradiation.
- Chemotherapy plus radiation therapy for cancer that recurs in the testicles only.
Some of the treatments being studied in clinical trials for recurrent childhood ALL include the following:
- A clinical trial of chemotherapy with stem cell transplant, using stem cells from a donor who is not related to the patient, with or without total-body irradiation.
- A clinical trial of new anticancer drugs and new combination chemotherapy treatments.
Information about these and other ongoing clinical trials is available from the NCI Web site.
Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood acute lymphoblastic leukemia.
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The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Editorial changes were made and images were added to this summary.
PDQ is a comprehensive cancer database available on NCI's Web site.
PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.
PDQ contains cancer information summaries.
The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.
The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.
Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.
PDQ also contains information on clinical trials.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." In the United States, about two-thirds of children with cancer are treated in a clinical trial at some point in their illness.
Listings of clinical trials are included in PDQ and are available online at NCI's Web site. Descriptions of the trials are available in health professional and patient versions. For additional help in locating a childhood cancer clinical trial, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.
The PDQ database contains listings of groups specializing in clinical trials.
The Children's Oncology Group (COG) is the major group that organizes clinical trials for childhood cancers in the United States. Information about contacting COG is available on the NCI Web site or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.
The PDQ database contains listings of cancer health professionals and hospitals with cancer programs.
Because cancer in children and adolescents is rare, the majority of children with cancer are treated by health professionals specializing in childhood cancers, at hospitals or cancer centers with special facilities to treat them. The PDQ database contains listings of health professionals who specialize in childhood cancer and listings of hospitals with cancer programs. For help locating childhood cancer health professionals or a hospital with cancer programs, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.