Childhood acute lymphoblastic leukemia (ALL) is a type of cancer in which the bone marrow
makes too many lymphocytes (a type of white blood cell).
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.
There are subgroups of childhood ALL.
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.
Family history and exposure to radiation may affect the
risk of developing childhood ALL.
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.
Possible signs of childhood ALL include fever and bruising.
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
Tests that examine the blood and bone marrow are used to detect (find) and diagnose
childhood ALL.
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.
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."
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.
Certain factors affect prognosis (chance of recovery) and treatment options.
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.
Stages of Childhood Acute Lymphoblastic Leukemia
Once childhood ALL has been diagnosed, tests are done to find
out if the cancer has spread to the central nervous system (brain and spinal
cord), testicles, or to other parts of the body.
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.
There are three ways that cancer spreads in the body.
When cancer cells spread outside the blood, a solid tumor may form. This process is
called metastasis. The three ways that cancer cells spread in the body are:
- Through the blood. Cancer cells travel through the blood, invade
solid tissues in the body, such as the brain or heart, and form a solid
tumor.
- Through the lymph system. Cancer cells invade the lymph system,
travel through the lymph vessels, and form a solid tumor in other parts of
the body.
- Through solid tissue. Cancer cells that have formed a solid tumor
spread to tissues in the surrounding area.
The new (metastatic) tumor is the same type of cancer as the
primary cancer. For example, if leukemia cells spread to the brain, the cancer
cells in the brain are actually leukemia cells. The disease is metastatic
leukemia, not brain cancer.
In childhood ALL, risk groups are used instead of stages.
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 Acute Lymphoblastic Leukemia
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.
Treatment Option Overview
There are different types of treatment for childhood acute lymphoblastic leukemia (ALL).
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.
Some clinical trials are open only to patients who have not started treatment.
Children with ALL should have their treatment planned by a
team of doctors with expertise in treating childhood leukemia.
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.
The treatment of childhood ALL usually has 3 phases.
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.
Three types of standard treatment are used:
Chemotherapy
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
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).
Chemotherapy with stem cell transplant
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.
New types of treatment are being tested in clinical trials.
This section describes treatments that are being studied in
clinical trials. It may not mention every new treatment being studied.
Information about clinical trials is available from the NCI Web site.
High-dose chemotherapy
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.
Other drug 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.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the
best treatment choice. Clinical trials are part of the cancer research process.
Clinical trials are done to find out if new cancer treatments are safe and
effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on
earlier clinical trials. Patients who take part in a clinical trial may receive
the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the
way cancer will be treated in the future. Even when clinical trials do not lead
to effective new treatments, they often answer important questions and help move
research forward.
Patients can enter clinical trials before, during, or after
starting their cancer treatment.
Some clinical trials only include patients who have not yet
received treatment. Other trials test treatments for patients whose cancer has
not gotten better. There are also clinical trials that test new ways to stop
cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. These have been retrieved
from NCI's clinical trials database.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to
find out the stage of the cancer may be repeated. Some tests will be repeated in
order to see how well the treatment is working. Decisions about whether to
continue, change, or stop treatment may be based on the results of these tests.
This is sometimes called re-staging.
Some of the tests will continue to be done from time to time
after treatment has ended. The results of these tests can show if your condition
has changed or if the cancer has recurred (come back). These tests are sometimes
called follow-up tests or check-ups.
Treatment Options for Childhood Acute Lymphoblastic Leukemia
For some types or stages of cancer, there may not be any trials
listed. Check with your doctor for clinical trials that are not listed here but
may be right for you.
Untreated Childhood Acute Lymphoblastic Leukemia
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.
Childhood Acute Lymphoblastic Leukemia Subgroups
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).
Recurrent Childhood 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.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical
Trials Registry that are now accepting patients with recurrent childhood acute
lymphoblastic leukemia. For more specific results, refine the search by using
other search features, such as the location of the trial, the type of treatment,
or the name of the drug. General information about clinical trials is available
from the NCI Web site.
For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. The call is free and a trained Cancer Information Specialist is available to answer your questions. Information about ongoing clinical trials is available from the NCI Web site
For more information from the NCI, please write to this address:
NCI Public Inquiries Office
Suite 3036A
6116 Executive Boulevard, MSC8322
Bethesda, MD 20892-8322
Source: National Institutes of Health; National Cancer Institute
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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 8/1/2008...#6213
