Leukemia, a cancer of the blood, is the most common type of cancer in children. The most common type of leukemia in children is acute lymphoblastic leukemia (also called acute lymphocytic leukemia, acute lymphoid leukemia, or ALL). In ALL, the normal cells in a child's bone marrow are overwhelmed and replaced by abnormal lymphoblasts, which are immature lymphocytes (a type of white blood cell).
Normal lymphocytes fight infections in the body. However, when a child has ALL, the lymphoblasts that fill the bone marrow are abnormal and do not develop into mature cells that can produce the antibodies needed to prevent and treat infection. These abnormal lymphoblasts also crowd out other normal cells in the bone marrow, preventing the production of red cells, many other types of normal white blood cells, and platelets (cells that help blood to clot). If the bone marrow is not functioning correctly, the child may experience the following:
Anemia occurs when the blood has too few red cells. Anemia can lead to fatigue, irritability, sleepiness, pallor (paleness), shortness of breath, and a rapid heart beat.
Bruising or bleeding from injuries may occur more easily, because the blood cannot clot normally when the platelet count is low.
Infection may occur more often if the blood has too few normal white blood cells. Many types of white blood cells are needed (for example, granulocytes, which are often called segmented cells or polys, as well as monocytes and lymphocytes) for normal, healthy functions.
The leukemic lymphoblasts may also collect in the child's lymph nodes and cause them to swell. Lymphoblasts may also invade other organs, including the spinal cord, brain, skin, liver, spleen, ovaries (in females), testicles (in males), and the spinal fluid.
This section is about ALL that occurs in children, sometimes called pediatric ALL. For information on adult acute lymphocytic leukemia, please visit Leukemia, Acute Lymphocytic (ALL).
ALL is the most common type of childhood cancer. In 2008, an estimated 2,655 children, between the ages of 0 to 19, in the United States in the United States will be diagnosed with ALL. It is most common in younger children, especially between two and seven years old. The five-year relative survival rate (the percentage of patients who survive at least five years after the cancer is detected, excluding those who die from other diseases) for children with ALL is more than 80%.
Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of cases of this type of cancer, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with childhood ALL. Because the survival statistics are measured in five-year (or sometimes one-year) intervals, they may not represent advances made in the treatment or diagnosis of this cancer.
Statistics adapted from the American Cancer Society
A risk factor is anything that increases a person's chance of developing cancer. Some risk factors can be controlled, such as smoking, and some risk factors cannot be controlled, such as age and family history. Although risk factors can influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do.
Although doctors don't know what causes most childhood cancers, some evidence indicates that certain genetic factors may play a role in ALL. Specifically, conditions wherein children are born with an abnormal immune system, such as Down syndrome, ataxia telangiectasia, and Bloom syndrome, may place them at a higher risk of developing leukemia. A child with an identical twin that develops ALL before age six has an increased risk of developing leukemia. If an identical twin develops leukemia within the first few months of life, the other twin will almost always develop the same leukemia. It is important to note, however, that in the vast majority of cases, there is no known cause or risk factor for why a child has leukemia.
Children with ALL often, but not always, experience the following symptoms. These symptoms may be similar to symptoms of other medical conditions. For example, the early signs of ALL can look very much like the flu or other common illnesses, though often one or more of these symptoms persist for longer than one would expect. If you are concerned about a symptom on this list, please talk with your child's doctor.
Frequent infections
A fever that doesn't go away
Feeling weak and tired all the time
Aching bones and joints
Swollen lymph nodes (in the neck, under the arms, and groin, for example)
Pallor (paleness)
Bruising or bleeding easily
Difficulty breathing
The child's doctor may also detect an enlarged liver or spleen during a physical examination.
A patient history, physical examination, complete blood cell count (CBC), and bone marrow aspiration (see below) are the main procedures used to diagnose ALL, and each is used to establish a diagnosis of ALL or rule out other conditions (called differential diagnosis).
Blood tests. A complete blood count (CBC) will provide a count of each type of cell in the child's blood. The blood count may also reveal the presence of the abnormal leukemic cells. The blood count is abnormal, in some way, in nearly all children with ALL at the time of diagnosis.
Bone marrow aspiration. A bone marrow aspiration will be ordered if the blood test shows unusual blood counts or immature cells in the blood, or if there is a suspicion that a child may have leukemia. For this test, a health professional removes a sample of bone marrow from the child's hip with a needle and examines the sample under a microscope. This will tell the doctor whether the child has leukemia and, if so, what type of leukemia it is. The doctor or nurse will collect multiple samples of bone marrow at the same time for other tests, such as chromosome and molecular genetic analyses and immunophenotyping (see Classification). These additional tests may be critical in the determination of which treatment is most appropriate.
Lumbar puncture (spinal tap). A lumbar puncture will be performed to determine if the leukemia has spread to the spinal fluid. A needle is inserted through the child's back to remove a small sample of cerebrospinal fluid (CSF), the fluid that surrounds the brain and spinal cord. The CSF is examined under a microscope to determine if ALL is present. The presence or absence of leukemia in the central nervous system will determine, in part, the most appropriate treatment. There may be times when it is appropriate to deliver medicine to treat or prevent leukemia of the central nervous system at the same time that the diagnostic lumbar puncture is performed.
While there is no staging system for pediatric ALL, there are a number of factors that help determine the best treatment plan and determine a child's risk of recurrence (the cancer returning after treatment). Doctors plan treatment based on these and other factors:
Age of child at diagnosis. Infants younger than 12 months and children 10 years of age and older may require more intensive treatments.
White blood cell counts. Children with higher white blood cell counts may require more intensive treatments. Commonly, white blood cell counts are labeled as higher if they are more than 50,000/ml.
Immunophenotyping. This term refers to the profile of various proteins expressed by the leukemic cells. Knowing if the cancer cells express the proteins more closely resembling those of either normal B-cells or T-cells will help doctors plan appropriate treatment.
Genetic abnormalities in the leukemic cells. Abnormal numbers of chromosomes, abnormal structural changes in a chromosome, or certain molecular genetic changes in the chromosomes of leukemic cells may affect outcome and therefore treatment. Note that the genetic changes referred to here are changes in the genetic material of the leukemic cells, not the child's cells-the vast majority of children with leukemia have completely normal genetic material.
Response to early treatment. How the leukemia responds to the first one or two weeks of treatment (by examining the child's blood or bone marrow) may predict the ultimate response to treatment. Recent studies have shown that some children may need more intense treatment to improve the chance of a cure. This includes those whose cancer is not responding well to early treatment or those who have high levels of residual leukemic cells (cells remaining after treatment) at the end of remission induction (see Treatment ).
The following are used to describe the state of disease in children with ALL:
Untreated ALL. The child hasn't yet received any treatment.
ALL inremission. Normal levels of white and other blood cells have been restored after treatment. Physical examinations, blood counts, and bone marrow aspirates reveal no detectable leukemia. Remission is the absence of signs or symptoms of leukemia.
Recurrent ALL. The cancer has recurred (come back) after the child has had some period of remission. The leukemia may recur in the bone marrow, spinal fluid, testicle (for boys), or, far less commonly, in other areas of the body.
Refractory ALL. The leukemia has failed to go into remission, despite remission induction treatment (see below).
Clinical trials are the standard of care for the treatment of children with cancer. In fact, more than 60% of children with cancer are treated as part of a clinical trial. Clinical trials are research studies that compare standard treatments (the best treatments available) with newer treatments that may be more effective. Cancer in children is rare, so it can be hard for doctors to plan treatments unless they know what has been most effective in other children. Investigating new treatments involves careful monitoring using scientific methods, and all participants are followed closely to track progress.
To take advantage of these newer treatments, all children with cancer should be treated at a specialized cancer center. Doctors at these centers have extensive experience in treating children with cancer and have access to the latest research. Many times, a team of doctors treats a child with cancer. Pediatric cancer centers often have extra support services for children and their families, such as nutritionists, social workers, and counselors. Special activities for kids with cancer may also be available.
Three types of treatments are used to treat ALL in children: chemotherapy, radiation treatment, and high-dose chemotherapy followed by stem cell transplantation. Sometimes the treatments are used in combination.
Chemotherapy
Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. Chemotherapy is the primary treatment for ALL. Several drugs are used in chemotherapy for ALL. Chemotherapy may be given by mouth, injected into a vein or muscle, or injected into the CSF.
Chemotherapy is generally done in four phases:
Remission induction therapy uses chemotherapy to kill as many of the cancer cells as possible to cause the cancer to go into remission.
Central nervous system directed therapy kills any cancer cells in the central nervous system and prevents the spread of the cancer to the spinal fluid.
Consolidation therapy begins once the child's cancer has gone into remission. Higher doses of chemotherapy, or drugs not used during previous treatment sessions, are used to kill the majority of the remaining cancer cells.
Continuation or maintenance therapy lasts for two to three years to kill any residual leukemia.
Because chemotherapy attacks rapidly dividing cells, including those in normal tissues such as the hair, lining of the mouth, intestines, and bone marrow, children receiving chemotherapy may lose their hair, develop mouth sores, or have nausea and vomiting. In addition, chemotherapy may lower the body's resistance to infection, lead to increased bruising and bleeding, and cause fatigue. Less commonly, chemotherapy may cause learning problems and liver dysfunction. These side effects can be controlled during treatment and usually go away after chemotherapy is completed. The severity of the side effects depends on the type and amount of the drug being given and the length of time the child receives the drug. The degree to which children experience side effects may also be affected by other factors, including genetic differences in the way the medications are metabolized and the patient's overall health and well-being.
The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications you've been prescribed, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions through Cancer.Net's Drug Information Resources, which provides links to searchable drug databases.
Radiation therapy
Radiation therapy is the use of high-energy x-rays or other particles to kill cancer cells. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. Radiation therapy for ALL is generally used only when the cancer has spread to the brain, spinal fluid, or a boy's testicles, or in high-risk cases to help prevent the spread of leukemia to the spinal fluid. Radiation therapy is more often used for patients with T-cell leukemia.
Side effects from radiation therapy may include tiredness, mild skin reactions, upset stomach, and loose bowel movements. These side effects often go away soon after treatment is finished. However, long-term side effects of radiation treatment to the brain and body can occur and may potentially include learning disabilities and a risk of developing a brain tumor (see After Treatment).
Hematopoietic stem cell transplantation
Hematopoietic stem cells are special cells that can develop into different kinds of blood cells. Also called a bone marrow transplant, stem cell transplantation is generally offered only if a child has very high-risk disease or the leukemia returns after treatment. The doctor first gives high doses of chemotherapy, possibly along with radiation treatment, to destroy all of the child's bone marrow. The doctor then takes normal stem cells from bone marrow, an umbilical cord donor, or blood of a donor whose tissue matches the child's as closely as possible. The healthy stem cells are infused into the child's vein. They find their way into the bones and replace the destroyed bone marrow.
The best match for transplantation usually comes from a brother or sister. However, people who are not related to the child with ALL may also match, resulting in a successful transplantation.
Treatment for recurrent ALL depends on many factors, including the type of treatment the child received originally, the length of time between the initial diagnosis and the recurrence, and whether leukemic cells are found in the bone marrow, CSF, testicles, or in a combination of these sites at the time of recurrence. Doctors will plan treatment based on each child's circumstances; just as for newly diagnosed patients, clinical trials typically offer the best chance of cure.
Doctors and scientists are always looking for better ways to treat children with ALL. A clinical trial is a way to test a new treatment in order to prove that it is safe, effective, and possibly better than a standard treatment. Patients who participate in clinical trials are among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.
People decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that finding new drugs and other therapies is the only way to make progress in treating children with ALL. Even if they do not benefit directly from the clinical trial, their participation may benefit future children with ALL.
To join a clinical trial, patients must complete a learning process known as informed consent. During informed consent, the doctor should list all of the patient's options, so the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment. Learn more about clinical trials, including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and links to find cancer clinical trials.
Cancer and cancer treatment can cause a variety of side effects; some are easily controlled and others require specialized care. Below are some of the side effects that are more common to ALL and its treatments. For more detailed information on managing these and other side effects of cancer and cancer treatment, visit the Cancer.Net Managing Side Effects section.
Constipation. Constipation is the infrequent or difficult passage of stool. Constipation includes fewer bowel movements, stools that are abnormally hard, discomfort, or a feeling of incomplete rectal emptying. Patients with constipation may experience pain, swelling in the abdomen, loss of appetite, or nausea and/or vomiting. One of the drugs most commonly used to treat ALL, vincristine, often causes constipation.
Fatigue (tiredness). Fatigue is extreme exhaustion or tiredness. This side effect is significantly less common in children and adolescents than adults, but it can be very hard to manage. One of the most important aspects of dealing with fatigue is determining its cause(s). Depression, malnutrition and deconditioning can contribute to fatigue as can the child's therapy. Your child's doctors, nurses, child life workers, counselors, teachers and others will be able to help you determine the causes of your child's fatigue and the best approach to its therapy.
Hair loss (alopecia). A potential side effect of radiation therapy and chemotherapy is hair loss. Radiation therapy and chemotherapy cause hair loss by damaging the hair follicles responsible for hair growth. Hair loss may occur throughout the body, including the head, face, arms, legs, underarms, and pubic area. The hair may fall out entirely, gradually, or in sections. In some cases, the hair will simply thin-sometimes unnoticeably-and may become duller and dryer. Losing one's hair can be a psychologically and emotionally challenging experience and can affect a patient's self-image and quality of life. However, the hair loss is almost always temporary, and the hair grows back, commonly thicker and occasionally curlier than before, in the absence of radiation. Wigs and/or hats may be helpful while waiting for hair re-growth.
Infection. The most important side effect for you to be aware of it the increased risk of infection. An infection occurs when harmful bacteria, viruses, or fungi (such as yeast) invade the body and the immune system is not able to destroy them quickly enough. Patients with cancer are more likely to develop infections because both cancer and chemotherapy can weaken the immune system, most commonly by lowering the number of infection fighting white blood cells. Symptoms of infection include fever (temperature of 100.5°F or higher); listlessness, chills or sweating; sore throat or sores in the mouth; abdominal pain; pain or burning when urinating or frequent urination; diarrhea or sores around the anus; cough or breathlessness; redness, swelling, or pain, particularly around a cut or wound; and unusual vaginal discharge or itching. Even in the absence of fever, if your child is "not right", sleepy, with a poor appetite, please call your doctor or nurse to discuss whether or not your child should be evaluated for a potential infection.
Mouth sores (mucositis). Mucositis is an inflammation of the inside of the mouth and throat, leading to painful ulcers and mouth sores. It occurs in up to 40% of patients receiving chemotherapy treatments. Mucositis can be caused by a chemotherapy, the reduced immunity brought on by chemotherapy, and/or an infection. Therapy for mucositis will vary, depending on its cause, but you should contact your medical team if your child has significant pain or cannot eat or drink normally.
Nausea and vomiting. Vomiting, also called emesis or throwing up, is the act of expelling the contents of the stomach through the mouth. It is a natural way for the body to rid itself of harmful substances. Nausea is the urge to vomit. Though nausea and vomiting are common in patients receiving chemotherapy and in some patients receiving radiation therapy, the drugs used to treat ALL are less commonly associated with nausea and vomiting than those used to treat other cancers. When it is minor and treated quickly, nausea and vomiting can be quite uncomfortable but cause no serious problems. Persistent vomiting can cause dehydration, electrolyte imbalance, weight loss, depression, and avoidance of chemotherapy. Be sure to talk to your medical team about ways to prevent and treat nausea and vomiting.
Neutropenia. Neutropenia is an abnormally low level of neutrophils, a type of white blood cell. All white blood cells help the body fight infection. Neutrophils fight infection by destroying bacteria. Patients who have neutropenia are at increased risk for developing serious bacterial infections because there are not enough neutrophils to destroy harmful bacteria. Neutropenia is common in patients with leukemia.
Skin problems. The skin is an organ system that contains many nerves. Because of this, skin problems can be very painful. The treatment used for leukemia does not commonly cause skin problems with three significant exceptions: 1) Patients receiving chemotherapy are much more likely to develop sunburn. Be sure to use hats, sun-protective clothing, umbrellas, and sun screen lotion. 2) Teenagers may experience more severe acne when taking steroids, and; 3) Some (not all) chemotherapy can cause a severe burn, if they leak out of the intravenous (IV) tube into the skin. Many patients with leukemia have a catheter (a thin tube temporarily put into a large vein) placed to avoid or reduce this risk.
Thrombocytopenia. Thrombocytopenia is an unusually low level of platelets in the blood. Platelets, also called thrombocytes, are the blood cells that stop bleeding by plugging damaged blood vessels and helping the blood to clot. Patients with low levels of platelets bleed more easily and are prone to bruising. Thrombocytopenia caused by chemotherapy is usually temporary. There are times when platelet transfusions may be recommended.
After treatment for ALL ends, talk with your child's doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your child's recovery for the coming months and years. All children treated for cancer, including ALL, should have life-long, follow-up care.
Based on the type of treatment the child received, the doctor will determine what examinations and tests are needed to check for long-term side effects, such as learning disabilities, depression, anxiety, post-traumatic stress disorder, and the possibility of secondary cancers. Your doctor can recommend the necessary screening tests. Follow-up care should address the child's quality of life, including any developmental or emotional concerns. Learn more about Late Effects of Childhood Cancer.
For the majority of childhood ALL survivors, there are relatively few significant long-term side effects. A recent study demonstrated that patients who did not receive radiation therapy and have been in remission at least 10 years were similar to the general population in terms of rates of health-care coverage, marriage, and employment. Survivors should be sensitive to their level of functioning, level of stress, and may need counseling if any problems arise. It is critical that children with school problems undergo neuropsychological testing in order to determine their cause. Tutoring in academic subjects or providing help in achieving appropriate organizational skills is very important, and any problems are correctable with help.
If a child did not receive radiation therapy and intensive chemotherapy with cyclophosphamide (Cytoxan, Neosar) and/or etoposide (VePesid), second cancers and infertility (the inability to have children) are rare. Also, future heart problems are rare, as the total dose of anthracycline (a type of antibiotic) given in the majority of pediatric ALL clinical trials is less than 250 mg/m2. Though the risk to any one individual is small, radiation therapy increases the risk of secondary brain tumors, as well as the risk of learning disabilities.
Patients should be followed at gradually increasing intervals, throughout their lifetime, to monitor for:
Recurrence (rare after the end of the second year), after chemotherapy is completed
Liver disease, caused by chemotherapy or transfusion-related infection (both very rare)
School problems (as described above)
Problems related to obtaining insurance coverage
Note that much of the data that will be used to demonstrate that childhood cancer survivors are good candidates for insurance will come through the collection of long-term, follow-up data on survivors.
The child's family is encouraged to organize and keep a record of the child's medical information, so that as the child enters adulthood, he or she has a clear, written history of the diagnosis and details of the treatment given. The doctor's office can help you compile this, and it should include recommendations from the doctor about the schedule for follow-up care. This information will be valuable to doctors who care for your child during his or her lifetime.
Children who have had cancer can also enhance the quality of their future by following established guidelines for good health into and through adulthood, including not smoking, maintaining a healthy weight, eating a balanced diet, and participating in regular physical activity. Talk with the doctor about developing a plan that is best for your child's needs.
Research involving more advanced diagnostic procedures and treatment for childhood ALL is ongoing. The following advancements may still be under investigation in clinical trials and may not be approved or available at this current time. Always discuss all diagnostic and treatment options with your doctor.
New methods for detecting small numbers of leukemic cells, too small to see under a microscope, were introduced in the 1990s. These techniques are now being used in order to detect minimal residual disease (MRD) following remission. There are times when measurements of MRD, in blood or bone marrow samples, will be used to determine your child's risk level and treatment. Much work is ongoing with respect to what level of MRD, measured at which time point, affects the rate of recurrence and whether changes in treatment will impact the meaning of these measurements and their impact on ultimate cure. Current investigations are studying the relationship between the biology of the leukemic cells and these measurements.
Finding new ways to treat leukemia are important areas of research. New cytotoxic agents, such as the T-cell-specific drug nelarabine (Arranon), and the antibody epratuzamab are under investigation and may improve the cure rate for children with ALL in the future. Other new agents-antibodies, cytotoxic drugs, and other biologically active agents-are being explored with the hope that they will increase the cure rate and/or decrease the likelihood that side effects will occur.
Regular communication with your child's doctor is important in making informed decisions about your child's health care. Consider asking the following questions of your child's doctor:
What type of leukemia does my child have?
Will more tests be needed to confirm the diagnosis?
What are the results of the analysis of the spinal fluid? What does this mean?
What are the results of the bone marrow aspiration? What does this mean?
What are the results of the cytogenetic studies done? What does this mean?
What are the treatment options?
What clinical trials are open for my child?
What treatment option do you recommend? Why?
What side effects may occur, both in the short term and in the long term?
What follow-up tests will be needed, and how often?
American Society for Blood and Marrow Transplantation
85 W Algonquin Rd., Ste. 550
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Phone: 847-427-0224 www.asbmt.org
Blood and Marrow Transplant Information Network
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Candlelighters Childhood Cancer Foundation
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Kensington, MD 20895
Toll Free: 800-366-2223
Phone: 301-962-3520 www.candlelighters.org
Make-A-Wish Foundation
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National Bone Marrow Transplant Link
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Phone: 248-358-1886
Toll Free: 800-LINK-BMT (800-546-5268) www.nbmtlink.org
National Childhood Cancer Foundation
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National Children's Cancer Society 1015 Locust, Ste. 600
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Toll Free: 800-532-6459
Phone: 314-241-1600 www.nationalchildrenscancersociety.org
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Pat. Adv.: 888-999-6743 www.marrow.org
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