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300 Longwood Avenue
Boston, MA 02115
(617) 355-6000
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My Child Has:
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Acute Lymphoblastic Leukemia
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Leukemia is cancer of the blood. It develops in the bone marrow, the soft, spongy center of the long bones that produces the three major blood cells: white blood cells to fight infection; red blood cells that carry oxygen; and platelets that help blood clot and stop bleeding.
When a child has leukemia, the bone marrow, for an unknown reason, begins to make white blood cells that do not mature correctly, but continue to reproduce themselves. Normal, healthy cells only reproduce when there is enough space for them to fit. The body can regulate the production of cells by sending signals when to stop. With leukemia, these cells do not respond to the signals to stop and reproduce, regardless of space available.
These abnormal cells reproduce very quickly and do not function as healthy white blood cells to help fight infection. When the immature white blood cells, called blasts, begin to crowd out other healthy cells in the bone marrow, the child experiences the symptoms of leukemia (i.e., infections, anemia, bleeding).
Acute lymphoblastic leukemia (ALL), also called lymphocytic or lymphoid, accounts for about 75 to 80 percent of the childhood leukemias. In this form of the disease, the lymphocyte cell line is affected. The lymphocytes, a type of white blood cell, normally fight infection. With acute lymphocytic leukemia, the bone marrow makes too many of these lymphocytes and they do not mature correctly. The lymphocytes overproduce, thus crowding out other blood cells. In addition, these immature blood cells (blasts) do not work properly to fight infection. In some cases, these cancerous lymphocytes can affect the brain, spinal cord and other organs.
Lymphocytes are carried throughout the body in a colorless fluid called lymph. The lymph system is a network of thin tubes that reach into all parts of the body, like blood vessels. Lymph nodes are the small groups of organs found along the lymph system branches. Lymph nodes are found in the underarm, pelvis, neck and abdomen. The spleen (an organ in the upper abdomen that makes lymphocytes and filters old blood cells from the blood), the thymus (a small organ beneath the breastbone), and the tonsils (an organ in the throat) are also part of the lymph system.
Acute leukemia can occur over a short period of days to weeks.
As you read further below, you will find general information about ALL. If you would like to view summary information about cancer first, see the cancer overview.
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Leukemia is the most common form of cancer in childhood. It affects approximately 3,000 children each year in the US, accounting for about 30 percent of childhood cancers. Leukemia can occur at any age, although it is most commonly seen in children between 2 and 6 years of age. The disease occurs slightly more frequently in males than in females, and is more commonly seen in Caucasian children than in African-American children, or children of other races.
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In nearly all cases, it is not known what causes leukemia. The majority of childhood leukemias are acquired genetic diseases. This means that gene mutations and chromosome abnormalities in leukemia cells occur sporadically (by chance). The abnormalities found in leukemia cells are not found in the other cells of the body.
The immune system plays an important role in protecting the body from diseases, and possibly cancer. An alteration or defect in the immune system may increase the risk for developing leukemia. Factors such as exposure to certain viruses, environmental factors, chemical exposures, and various infections have been associated with damage to the immune system, but none of these factors has been definitively linked as a cause of childhood leukemia.
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In addition to acute lymphoblastic leukemia (ALL), there are two other main types of leukemia:
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- acute myelogenous leukemia (AML) - also called granulocytic, myelocytic, myeloblastic, or myeloid, accounts for about 20 percent of the childhood leukemias. Acute myelogenous leukemia is a cancer of the blood in which too many granulocytes, a type of white blood cell, are produced in the marrow. The granulocytes normally fight infection. With acute myelogenous leukemia, the bone marrow makes too many of these cells and they do not mature correctly. The granulocytes overproduce, thus, crowding out other blood cells. Immature blood cells (blasts) do not work properly to fight infection. Acute leukemia can occur over a short period of days to weeks. Children with certain genetic syndromes, including Fanconi anemia, Bloom syndrome, Kostmann syndrome, and Down syndrome, are at a higher risk of developing AML than other children.
It is thought that lymphoblastic and myelogenous leukemias differ based upon the stage of development of the cell in which the leukemia arises. The pluripotent stem cell is the first stage of development of all of the blood cells (white blood cells, red blood cells and platelets). This stem cell goes through stages of development until it matures into a functioning cell.
The type of leukemia (lymphoblastic or myelogenous) is determined by where the cell is in its development when it becomes malignant, or cancerous. Early in its development, the stem cell becomes either a lymphocyte precursor cell or a myeloid precursor cell. The lymphoid cells mature into either B-cell precursor or T-cell ALL, depending on the type of lymphoid precursor cell. If the leukemia is found even further along this stage of development, it is sometimes referred to as a mature B-cell or T-cell ALL.
The myeloid cells develop into platelets, red blood cells and specialized white blood cells called neutrophils and macrophages. There are many classifications of AML. The type of leukemia is determined by the stage of development when the normal cells become leukemia cells.
- chronic myelogenous leukemia (CML) - is uncommon in children. Chronic myelogenous leukemia is cancer of the blood in which too many granulocytes, a type of white blood cell, are produced in the marrow. The granulocytes normally fight infection. With this disease, the bone marrow makes too many of these cells and they do not mature correctly. The marrow continues to produce these abnormal cells which crowd out other healthy blood cells. Chronic myeloblastic leukemia can occur over a period of months or years. A specific chromosome rearrangement is found in patients with CML. Part of chromosome #9 breaks off and attaches itself to chromosome #22, so that there is an exchange of genetic material between these two chromosomes. This rearrangement changes the position and functions of certain genes, which results in uncontrolled cell growth. Other chromosome abnormalities can also be present.
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Because leukemia is cancer of the blood-forming tissue called the bone marrow, the initial symptoms are often related to abnormal bone marrow function. The bone marrow is responsible for storing and producing about 95 percent of the body's blood cells, including the red blood cells, white blood cells, and platelets.
When leukemia occurs, the abnormal white blood cells (blasts) begin to reproduce very rapidly and begin crowding out and competing for nutrients and space with the other healthy cells. The following are the most common symptoms of leukemia. However, each child may experience symptoms differently. Symptoms may include:
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- anemia - when red blood cells are unable to be produced because of the crowding in the marrow, anemia is present. With anemia, the child may appear tired, pale, and may breathe faster to compensate for the decrease in oxygen carrying capacity. The number of red blood cells on a blood count will be below normal.
- bleeding and/or bruising - when platelets are unable to be produced because of the crowding in the marrow, bleeding can occur and the child may begin to bruise more easily. Petechia are tiny red dots often seen on the skin of a child with low number of platelets. Petechia are very small blood vessels that have "leaked" or bled. The number of platelets on a blood count will be below normal. Thrombocytopenia is the term used for a decreased number of platelets.
- recurrent infections - although there may be an unusually high number of white blood cells on a blood count of a child with leukemia, these white blood cells are immature and do not fight infection. The child may have had repetitive viral or bacterial infections over the past few weeks. The child with leukemia often shows symptoms of an infection such as fever, runny nose, and cough.
- bone and joint pain - pain in bones and joints is another common symptom of leukemia. This pain is usually a result of the bone marrow being overcrowded and "full."
- abdominal distress - abdominal pain may also be a symptom of leukemia. Leukemia cells can collect in the kidney, liver, and spleen, causing enlargement of these organs. Pain in the abdomen may cause a child to have loss of appetite and weight loss.
- swollen lymph nodes - the child may also have swelling in the lymph nodes under the arms, in the groin, chest, and in the neck. Lymph nodes are responsible for filtering the blood. Leukemia cells may collect in the nodes, causing swelling.
- difficulty breathing (dyspnea) - with T-cell ALL, these leukemia cells tend to clump together around the thymus. This mass of cells present in the middle of the chest can cause pain and difficulty breathing (dyspnea). Wheezing, coughing, and/or painful breathing requires immediate medical attention.
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With acute leukemia (ALL or AML), these symptoms may occur suddenly in a matter of days or weeks.
It is important to understand that the symptoms of leukemia may resemble other blood disorders or medical problems. These are common symptoms of the disease, but do not include all possible symptoms. Children may experience symptoms differently. Always consult your child's physician for a diagnosis.
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In addition to a complete medical history and physical examination, diagnostic procedures for leukemia may include:
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- bone marrow aspiration and biopsy - marrow may be removed by aspiration or a needle biopsy under local anesthesia. In aspiration biopsy, a fluid specimen is removed from the bone marrow. In a needle biopsy, marrow cells (not fluid) are removed. These methods are often used together. Most often samples are obtained from the bones of the pelvis (upper buttocks).
- complete blood count (CBC) - a measurement of size, number, and maturity of different blood cells in a specific volume of blood
- additional blood tests - may include blood chemistries, evaluation of liver and kidney functions, and genetic studies
- computerized tomography scan (also called a CT or CAT scan) - a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.
- magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body
- x-ray - a diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film
- ultrasound (also called sonography) - a diagnostic imaging technique that uses high-frequency sound waves and a computer to create images of blood vessels, tissues, and organs. Ultrasounds are used to view internal organs as they function, and to assess blood flow through various vessels.
- lymph node biopsy - surgical removal of lymph node tissue to examine under a microscope to look for the presence of cancer cells
- spinal tap/lumbar puncture - a special needle is placed into the lower back, into the spinal canal. This is the area around the spinal cord. The pressure in the spinal canal and brain can then be measured. A small amount of cerebral spinal fluid (CSF) can be removed and sent for testing to determine if there is an infection or other problems. CSF is the fluid that bathes your child's brain and spinal cord.
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Specific treatment for leukemia will be determined by your child's physician based on:
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- your child's age, overall health, and medical history
- the extent of the disease
- your child's tolerance for certain medications, procedures, or therapies
- how your child's physician expects the disease to progress
- your opinion or preference
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Treatment usually begins by addressing the presenting symptoms such as anemia, bleeding, and/or infection. In addition, treatment for leukemia may include (alone or in combination) the following:
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- chemotherapy - a drug treatment that works by interfering with the cancer cell's ability to grow or reproduce. Different groups of drugs work in different ways to fight cancer cells and shrink tumors. Chemotherapy may be used alone for some types of cancer or in conjunction with other therapy such as radiation. Often, a combination of chemotherapy drugs is used to fight a specific cancer. Certain chemotherapy drugs may be given in a specific order depending on the type of cancer it is being used to treat. While chemotherapy can be quite effective in treating certain cancers, the agents do not differentiate normal healthy cells from cancer cells. Because of this, there can be many adverse side effects during treatment. Being able to anticipate these side effects can help the care team, parents, and child prepare, and, in some cases, prevent these symptoms from occurring, if possible.
Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells. Chemotherapy can be given:
- as an injection into the muscle or fat tissue
- intravenously (directly to the bloodstream; also called IV)
- intrathecally - chemotherapy given directly into the spinal column with a needle
- radiation therapy - using high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors
- stem cell transplant - a treatment that is almost never used to treat ALL, except for certain rare subtypes or if the leukemia comes back after initial treatment with chemotherapy (and sometimes radiation). Stem cell transplant is a treatment involving stem cells, a specific type of cell from which all blood cells develop. Stem cells develop into red blood cells to carry oxygen, white blood cells to fight disease and infection, and platelets to aid in blood clotting. Stem cells are found primarily in the bone marrow, but some also circulate in the blood stream.
Treatment may begin with high-dose chemotherapy (perhaps combined with radiation therapy) to destroy all of the bone marrow, stem cells and cancerous cells, in the body. Then, healthy stem cells from another person are used to help restore normal blood production in the patient with ALL.
The use of stem cells from another individual whose tissue is the same as, or almost the same as, the patient's is called allogeneic transplantation.
Stem cells collected from patients themselves prior to intensive treatment can also be used to supplement the recovery of the patient's own cells after particularly aggressive course of chemotherapy or radiation therapy. Through this procedure, stem cells are taken from the patient, treated in a laboratory to kill any remaining cancer cells, and kept in frozen storage for later use. After chemotherapy and/or radiation therapy, the saved stem cells are thawed and given back to the patient to grow new bone marrow and other stem cells. The use of a patient's own cells is referred to as autologous transplantation.
- other medications - to prevent or treat damage to other systems of the body caused by leukemia treatment; or for nausea and side effects of treatment
- blood transfusions - red blood cells, platelets
- antibiotics - to prevent/treat infections
- continuous follow-up care - to determine response to treatment, detect recurrent disease, and manage late effects of treatment
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There are various stages in the treatment of leukemia, including the following:
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- induction - a combination of chemotherapy and medications given to stop the process of abnormal cells being made in the bone marrow. Remission is the goal in this stage of treatment. Remission means the leukemia cells are no longer visible under the microscope when examining either the bone marrow or blood. However, even when remission is achieved, leukemia is still present in the body, which is why further therapy is necessary. The induction phase may last approximately one month and can be repeated if remission is not achieved.
- intensification or consolidation - continued treatment with chemotherapy to kill leukemia cells. Even though leukemia cells are not visible in a blood test or bone marrow examination, they are still present in the body. Therapy in this phase may include chemotherapy given to the central nervous system to kill any leukemia cells present there, or to prevent the spread of leukemia cells to the spinal cord or brain. Radiation therapy may also be given to the brain during this phase.
- maintenance or continuation - the goal of this stage is to eradicate all leukemia from the body. Usually, less intensive chemotherapy is used, much of which can be given at home. This phase can last months to several years. Regular visits to your child's physician are required in order to determine response to treatment, detect any recurrent disease, and manage any side effects of treatment.
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Relapse may occur even with aggressive therapy. Relapse is when the bone marrow begins making abnormal cells again. Relapse can occur during any of the stages of treatment, or may occur months or years after treatment has ended.
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Prognosis greatly depends on:
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- the extent of the disease.
- disease response to treatment.
- genetic abnormalities of the leukemia.
- age and overall health of the child.
- your child's tolerance of specific medications, procedures, or therapies.
- new developments in treatment.
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As with any cancer, prognosis and long-term survival can vary greatly from child to child. Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow-up care is essential for the child diagnosed with leukemia. Side effects of radiation and chemotherapy, as well as second malignancies, can occur in survivors of leukemia. New methods are continually being discovered to improve treatment and to decrease side effects of the treatment for the disease.
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At Children's Hospital/Dana-Farber Cancer Institute, researchers have been conducting randomized clinical studies in childhood ALL for nearly 30 years. CH/DFCI is recognized as an international leader in the treatment of this disease. CH/DFCI clinicians design and use their own protocols and have recently reported the best cure rates for this disease yet published.
CH/DFCI scientists have identified new genetic subtypes of ALL and are investigating how these various subtypes respond to therapy. The goal is to reduce treatment intensity for leukemia with good outcomes (to avoid side effects) and testing more intense or newer therapies for subtypes with poorer outcomes (to improve cure rates). These researchers have conducted extensive research to reduce the side effects of chemotherapy and radiation therapy. Efforts include testing new supportive care medications to protect the heart from chemotherapy, and investigating new ways of treating the central nervous system, such as reducing the dose or eliminating cranial radiation to avoid learning problems later in life.
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The information on this website should not be taken as medical advice,
which can only be given to you by your personal health care professional. |
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Copyright © Children's Hospital Boston. All rights reserved. |
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