What is childhood acute myeloid leukemia?

 

Childhood acute myeloid leukemia (AML) is a cancer of the blood-forming tissue,primarily the bone marrow and lymph nodes. AML is also called acutenonlymphocytic leukemia or acute myelogenous leukemia, and is divided intoseveral subtypes. It is less common than acute lymphocytic leukemia (alsocalled acute lymphoblastic leukemia or ALL), another form of leukemia thatoccurs in children. Children with Down syndrome have an increased risk of AMLduring the first 3 years of life.

All types of blood cells are produced by the bone marrow. The bone marrow isthe spongy tissue inside the large bones of the body. The bone marrow makesred blood cells (which carry oxygen and other materials to all tissues of thebody), white blood cells (which fight infection), and platelets (which helpmake the blood clot).

The bone marrow produces new blood cells. In leukemia the bone marrow startsproducing large numbers of abnormal blood cells usually white blood cells. These abnormal, immature white blood cells are called blasts. These cellsflood the blood stream and lymph system, and may invade vital organs such asthe brain, testes, ovaries, or skin. Acute promyelocytic leukemia is a raretype of AML that prevents blood from clotting normally. In rare cases, AMLtumor cells form a solid tumor called an isolated granulocytic sarcoma orchloroma.

Leukemia can be acute (progressing quickly with many immature blasts) orchronic (progressing slowly with more mature-looking cancer cells). Acutemyeloid leukemia can occur in both children and adults. Treatment is differentfor adults than it is for children. (Refer to the PDQ summaries on AdultMyeloid Leukemia Treatment; Chronic Lymphocytic Leukemia Treatment; ChronicMyelogenous Leukemia Treatment; Adult Acute Lymphoblastic Leukemia Treatment;Childhood Acute Lymphoblastic Leukemia Treatment; and Hairy Cell LeukemiaTreatment for more information.)

Early signs of AML may be fever, chills, bleeding or bruising easily, swollenlymph nodes, and other symptoms similar to those of the flu, such as feelingweak or tired all the time, with aching bones or joints. If your child hassymptoms of leukemia, his or her doctor may order a blood test to count thenumber of cells and examine them under a microscope. If the results of theblood tests are not normal, a bone marrow biopsy may be performed. During thistest, a needle is inserted into a bone in the hip and a small amount of bonemarrow is removed and examined under a microscope, enabling the doctor todetermine what kind of leukemia your child has and plan the best treatment. Chromosomal analysis may also be performed. These additional tests can helpdistinguish AML from ALL and other leukemias and allow doctors to better plantreatment.



Other childhood myeloid malignancies

 

Myelodysplastic syndromes (MDS) are disorders of the blood-forming cells. Myelodysplastic syndromes usually cause a deficiency in white blood cells, redblood cells, and platelets, and may lead to AML.

Juvenile myelomonocytic leukemia (JMML) is an extremely rare cancer of theblood-forming cells. Children with neurofibromatosis 1 (NF1) are at anincreased risk for developing JMML.



Stage Explanation

 

There is no staging for acute myeloid leukemia (AML). AML is always spreadthroughout the bloodstream at the time of diagnosis, and sometimes invadesother body tissue. Patients are often grouped according to whether or not theyhave been previously treated for leukemia.



Untreated

 

Untreated AML means that no treatment has yet been given except to alleviate ortreat symptoms of the disease. There are too many white blood cells in theblood and bone marrow, and there may be other signs and symptoms of leukemia. In rare cases, AML tumor cells appear as a solid tumor called an isolatedgranulocytic sarcoma or chloroma.



In remission

 

Remission means that the numbers of white blood cells and other cells in theblood and bone marrow are approaching normal following initial treatment withchemotherapy and that there are no signs or symptoms of leukemia.



Recurrent/refractory

 

Recurrent means that the leukemia has come back (recurred) after going intoremission. Refractory means that the leukemia failed to go into remissionfollowing treatment.



Treatment Option Overview

 

How childhood acute myeloid leukemia (AML) is treated

 

There are treatments for all patients with childhood acute myeloid leukemia(AML). Experienced doctors working together may provide the best treatment forchildren with AML. Your child's treatment will often be planned by a team ofchildhood cancer specialists with experience and expertise in treatingleukemias of childhood.

The primary treatment for AML is chemotherapy, sometimes followed by bonemarrow transplantation. Radiation therapy may be used in certain cases. Biological therapy is also being studied in clinical trials.

Chemotherapy is the use of drugs to kill cancer cells. Chemotherapy drugs maybe taken by mouth or injected into a vein (intravenous injection) or a muscle. Chemotherapy is called a systemic treatment because the drug enters thebloodstream, travels through the body, and can kill cancer cells throughout thebody. Chemotherapy may sometimes be injected into the fluid that surrounds thebrain and spinal cord (intrathecal chemotherapy).

Radiation therapy uses x-rays or other high-energy rays to kill cancer cellsand shrink tumors. Radiation for AML usually comes from a machine outside thebody (external radiation therapy).

Bone marrow transplantation, a newer type of treatment, is used to replace thepatient's bone marrow with healthy bone marrow. First, high doses ofchemotherapy with or without radiation therapy are given to destroy all of thebone marrow in the body. Healthy marrow is then taken from another person (adonor) whose tissue is the same as or almost the same as the patient's. Thedonor may be a twin (the best match), a brother or sister, or another personnot related. The healthy marrow from the donor is given to the patient througha needle in a vein, and the healthy marrow replaces the marrow that wasdestroyed. A bone marrow transplant using marrow from a relative or person notrelated is called an allogeneic bone marrow transplant.

Another type of bone marrow transplant, called autologous bone marrowtransplant, may be used. During this procedure, bone marrow is taken from thepatient and may be treated with drugs to kill any cancer cells. The marrow isthen frozen to save it. Next, the patient is given high-dose chemotherapy,with or without radiation therapy, to destroy all of the remaining marrow. Thefrozen marrow that was saved is then thawed and returned to the patient toreplace the marrow that was destroyed.

Biological therapy attempts to stimulate or restore the ability of thepatient's immune system to fight cancer. It uses substances produced by thepatient's own body or made in a laboratory to boost, direct, or restore thebody's natural defenses against disease. Biological therapy is sometimescalled biological response modifier therapy or immunotherapy.

Treatment for AML is ordinarily divided into 2 phases: induction andconsolidation. A third phase, intensification, may also be used. Duringinduction therapy, chemotherapy is used to kill as many of the leukemia cellsas possible and cause the leukemia to go into remission. Once the leukemiagoes into remission and there are no signs of leukemia, consolidation therapyis given. The purpose of postremission therapy (consolidation andintensification) is to kill any remaining leukemia cells. Your child mayreceive either or both phases of postremission therapy.

As preventive therapy, your child may also receive central nervous system (CNS)prophylaxis, which consists of intrathecal and/or high-dose systemicchemotherapy to the central nervous system (CNS) to kill any leukemia cellspresent there, or to prevent the spread of cancer cells to the brain and spinalcord even if no cancer has been detected there. Radiation therapy to the brainmay also be given, in addition to chemotherapy, for this purpose.

Unwanted side-effects can result from treatment long after it ends, so it is important that your child continue to be seen by his or her doctor. Chemotherapy can lead to heart, kidney, and hearing problems after treatment isfinished. Radiation therapy may cause problems with growth and development.

Treatment for childhood AML depends on whether or not the patient has beenpreviously treated for leukemia and the type of leukemia. The best treatmentis given by cancer doctors with experience in treating children with leukemia,and is given at hospitals where leukemia patients are often treated.

Your child may receive treatment that is considered standard based on itseffectiveness in a number of patients in past studies, or you may choose tohave your child take part in a clinical trial. Not all patients are cured withstandard therapy, and some standard treatments may have more side effects thanare desired. For these reasons, clinical trials are designed to test newtreatments and to find better ways to treat cancer patients, and are based onthe most up-to-date information. Clinical trials are ongoing in most parts ofthe country for most types of childhood AML. For more information, call theCancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at1-800-332-8615.



Untreated Childhood Acute Myeloid Leukemia and Other Childhood Myeloid Malignancies

 

Your child may receive treatment that is considered standard based on itseffectiveness in a number of patients in past studies, or you may choose tohave your child take part in a clinical trial. Not all patients are cured withstandard therapy, and some standard treatments may have more side effects thanare desired. For these reasons, clinical trials are designed to test newtreatments and to find better ways to treat cancer patients, and are based onthe most up-to-date information. Clinical trials are ongoing in most parts ofthe country for most types of childhood AML. For more information, call theCancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at1-800-332-8615



Childhood acute myeloid leukemia

 

Your child's treatment will probably be induction chemotherapy using 2 or morechemotherapy drugs to kill cancer cells and cause the leukemia to go intoremission. Induction chemotherapy is usually successful in inducing remission. Intrathecal chemotherapy with or without radiation therapy to the brain may begiven to prevent the spread of cancer cells to the brain and spinal cord. Biological therapy may be added to treatment to help your child recover morequickly from the side effects of induction therapy.



Acute promyelocytic leukemia

 

Treatment for acute promyelocytic leukemia (APL) may include all-trans retinoicacid (ATRA) combined with chemotherapy. Arsenic trioxide is also being studiedin children with APL.



 

Myelodysplastic syndrome

 

Treatment for myelodysplastic syndromes may include chemotherapy followed bybone marrow or peripheral stem cell transplantation.



Juvenile myelomonocytic leukemia

 

Treatment for children with juvenile myelomonocytic leukemia may be peripheralstem cell transplantation, cis-retinoic acid, and chemotherapy.



Childhood Acute Myeloid Leukemia in Remission

 

Treatment will consist of additional chemotherapy or bone marrowtransplantation. Central nervous system prophylaxis and/or maintenancechemotherapy may also be given in some cases.



Recurrent Childhood Acute Myeloid Leukemia

 

Treatment depends on the type of treatment your child received before. You maywant to consider entering your child into a clinical trial. Treatmentscurrently being studied in clinical trials include new chemotherapy drugs, bonemarrow transplantation, and biological therapy. Treatment for recurrent acutepromyelocytic leukemia (APL) may consist of arsenic trioxide or regimensincluding all-trans retinoic acid.

Unwanted side effects can result from treatment long after it ends, so it isimportant that your child continue to be seen by his or her doctor. Chemotherapy can later lead to heart problems, as well as kidney and hearingproblems. Radiation therapy may interfere with a child's growth and mayincrease the risk of hormonal dysfunction and cataract formation.



To Learn More

Call

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.

Web sites and Organizations

The NCI's Cancer.gov Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. There are also many other places where people can get materials and information about cancer treatment and services. Local hospitals may have information on local and regional agencies that offer information about finances, getting to and from treatment, receiving care at home, and dealing with problems associated with cancer treatment.

Publications

The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

LiveHelp

The NCI's LiveHelp service, a program available on several of the Institute's Web sites, provides Internet users with the ability to chat online with an Information Specialist. The service is available from 9:00 a.m. to 10:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.

Write

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



About PDQ

 

PDQ is a comprehensive cancer database available on Cancer.gov.

PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at Cancer.gov, the 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.

In the United States, about two-thirds of children with cancer are treated in a clinical trial at some point in their illness. 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 new treatments, the risks involved, and how well they do or do not work. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard."

Listings of clinical trials are included in PDQ and are available online at Cancer.gov. 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 Cancer.gov 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.