General Information About Adult Acute Lymphoblastic Leukemia

Adult acute lymphoblastic leukemia (ALL) is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell).

Adult acute lymphoblastic leukemia (ALL; also called acute lymphocytic leukemia) is a cancer of the blood and bone marrow. This type of cancer usually gets worse quickly if it is not treated.

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.

Previous chemotherapy and exposure to radiation may increase the risk of developing 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:

• Being male.
• Being white.
• Being older than 70.
• Past treatment with chemotherapy or radiation therapy.
• Exposure to atomic bomb radiation.
• Having a certain genetic disorder such as Down syndrome.

Possible signs of adult ALL include fever, feeling tired, and easy bruising or bleeding.

The early signs of ALL may be similar to the flu or other common diseases. A doctor should be consulted if any of the following problems occur:

• Weakness or feeling tired.
• Fever.
• Easy bruising or bleeding.
• Petechiae (flat, pinpoint spots under the skin caused by bleeding).
• Shortness of breath.
• Weight loss or loss of appetite.
• Pain in the bones or stomach.
• Pain or feeling of fullness below the ribs.
• Painless lumps in the neck, underarm, stomach, or groin.

These and other symptoms may be caused by adult acute lymphoblastic leukemia or by other conditions.

Tests that examine the blood and bone marrow are used to detect (find) and diagnose adult 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): A procedure in which a sample of blood is drawn and checked for the following:
  • The number of red blood cells, white blood cells, and platelets.
  • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
  • The portion of the blood sample made up of red blood cells.
  Certain factors affect prognosis (chance of recovery) and treatment options.

  The prognosis (chance of recovery) and treatment options depend on the following:

  • The age of the patient.
  • Whether the cancer has spread to the brain or spinal cord.
  • Whether the Philadelphia chromosome is present.
  • Whether the cancer has been treated before or has recurred (come back).

  Stages of Adult Acute Lymphoblastic Leukemia

  Once adult ALL has been diagnosed, tests are done to find out if the cancer has spread to the central nervous system (brain and spinal cord) or to other parts of the body.

  The extent or spread of cancer is usually described as stages. It is important to know whether the leukemia has spread outside the blood and bone marrow in order to plan treatment. The following tests and procedures may be used to determine if the leukemia has spread:

  • 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.
  • 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.There is no standard staging system for adult ALL.

    The disease is classified as untreated, in remission, or recurrent.

    Untreated adult ALL

    The ALL is newly diagnosed and has not been treated except to relieve symptoms such as fever, bleeding, or pain.

    • The complete blood count is abnormal.
    • More than 5% of the cells in the bone marrow are blasts (leukemia cells).
    • There are signs and symptoms of leukemia.

    Adult ALL in remission

    The ALL has been treated.

    • The complete blood count is normal.
    • Less than 5% of the cells in the bone marrow are blasts (leukemia cells).
    • There are no signs or symptoms of leukemia in the brain and spinal cord or elsewhere in the body.

    Recurrent Adult Acute Lymphoblastic Leukemia

    Recurrent adult acute lymphoblastic leukemia (ALL) is cancer that has recurred (come back) after going into remission. The ALL may come back in the blood, bone marrow, or other parts of the body.

    Treatment Option Overview

    There are different types of treatment for patients with adult ALL.

    Different types of treatment are available for patients with adult 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.

    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.

    The treatment of adult ALL usually has 2 phases.

    The treatment of adult ALL is done in phases:

    • Remission 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.
    • Maintenance therapy: This is the second phase of treatment. It begins once the leukemia is in remission. The purpose of maintenance therapy is to kill any remaining leukemia cells that may not be active but could begin to regrow and cause a relapse. This phase is also called remission continuation therapy.

    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.

    Four 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 chemotherapy), 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 and stage of the cancer being treated.

    Intrathecal chemotherapy may be used to treat adult 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 adult 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.

    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 the patient or 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.

    Tyrosine kinase inhibitor therapy

    Anticancer drugs called tyrosine kinase inhibitors are used to treat some types of adult ALL. These drugs block the enzyme, tyrosine kinase, that causes stem cells to develop into more white blood cells (blasts) than the body needs. Two of the drugs used are imatinib mesylate (Gleevec) and dasatinib.

    New types of treatment are being tested in clinical trials. These include the following:Biologic therapy

    Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy.

    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.

    Treatment Options for Adult Acute Lymphoblastic Leukemia

    Untreated Adult Acute Lymphoblastic Leukemia

    Standard treatment of adult acute lymphoblastic leukemia (ALL) during the remission induction phase includes the following:

    • Combination chemotherapy.
    • CNS prophylaxis therapy including chemotherapy (intrathecal and/or systemic) with or without radiation therapy to the brain.

    Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with untreated adult acute lymphoblastic leukemia.

    Adult Acute Lymphoblastic Leukemia in Remission

    Standard treatment of adult ALL during the maintenance phase includes the following:

    • Combination chemotherapy with or without tyrosine kinase inhibitor therapy.
    • Chemotherapy with stem cell transplant.
    • CNS prophylaxis therapy including chemotherapy (intrathecal and/or systemic) with or without radiation therapy to the brain.

    Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with adult acute lymphoblastic leukemia in remission.

    Recurrent Adult Acute Lymphoblastic Leukemia

    Standard treatment of recurrent adult ALL may include the following:

    • Combination chemotherapy followed by stem cell transplant.
    • Low-dose radiation therapy as palliative care to relieve symptoms and improve the quality of life.
    • Tyrosine kinase inhibitor therapy.

    Some of the treatments being studied in clinical trials for recurrent adult ALL include the following:

    • A clinical trial of stem cell transplant using the patient's own stem cells.
    • A clinical trial of biologic therapy.
    • A clinical trial of new anticancer drugs.

    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 adult acute lymphoblastic leukemia.

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    Changes to This Summary (02/22/2008)

    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.

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