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Acute lymphocytic leukemia |
| Definition: |
| Acute lymphocytic leukemia is a progressive, malignant disease characterized by large numbers of immature white blood cells that resemble lymphoblasts. These cells can be found in the blood, the bone marrow, the lymph nodes, the spleen, and other organs. |
| Alternative Names: |
| ALL; Acute childhood leukemia; Cancer - acute childhood leukemia (ALL) |
| Causes, incidence, and risk factors: |
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Acute lymphocytic leukemia (ALL) is responsible for 80% of the acute leukemias of childhood, with the peak incidence occurring between ages 3 and 7. ALL also occurs in adults, where it comprises 20% of all adult leukemias. In acute leukemia, the malignant cell loses its ability to mature and specialize (differentiate) its function. These cells multiply rapidly and replace the normal cells. Bone marrow failure occurs as malignant cells replace normal bone marrow elements. The person becomes susceptible to bleeding and infection because the normal blood cells are reduced in number. Risk factors for acute leukemia include Down syndrome, a sibling with leukemia, and exposure to radiation, chemicals, and drugs. The incidence is 6 out of 100,000 people. |
| Symptoms: |
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| Signs and tests: |
Physical examination shows enlarged liver and spleen, bruising (ecchymosis) and evidence of bleeding (petechiae, purpura, and so on).
ALL may also alter the results of the following tests:
Classification of ALL now depends on a number of specific sophisticated tests, such as immunophenotyping, karyotyping, and terminal deoxynucleotidyltransferase (TdT) activity. The combined results of these tests allows pinpoint molecular diagnosis, which helps guide the treatment decisions, and clarify the likely prognosis. For instance, the cells of some leukemias contain chromosomal abnormalities. Those with the Philadelphia chromosome or with the t(4;11) translocation would tend to have a poor prognosis, thus intensive treatment and an early bone marrow transplant might be recommended preemptively. Other genes (such as the TEL/AML1 rearrangement) can indicate a very favorable prognosis. |
| Treatment: |
| The goal of treatment is remission of the cancer. A remission is achieved when the peripheral blood counts and the bone marrow are normal. Acute lymphocytic leukemia is treated with a combination of anti-cancer drugs (chemotherapy). A hospitalization of 3 to 6 weeks may be necessary for initial (induction) chemotherapy, however, subsequent chemotherapy sessions may be administered on an outpatient basis. Additionally, isolation procedures may be necessary if the lymphocyte count is very low to prevent exposure to infectious agents. Chemotherapy typically consists of a combination of 3 to 8 medications which may include: prednisone, vincristine, methotrexate, 6-mercaptopurine, and cyclophosphamide. It may also be necessary to administer blood products (e.g., packed red blood cells, platelets) to correct the anemia and low platelet count. Antibiotic therapy may be required to treat any secondary infections that develop. After remission is achieved, chemotherapy or radiation therapy is administered in the spinal column to treat any leukemic cells that may have invaded the spinal fluid. Subsequent therapy is directed at preventing relapse and consists of maintenance chemotherapy for up to one year . A bone marrow transplant after high-dose chemotherapy is a treatment option for cases that relapse or do not respond to other treatments. |
| Support Groups: |
| The stress of illness can often be helped by joining a support group where members share common experiences and problems. See cancer - support group and leukemia - support group. |
| Expectations (prognosis): |
| The probable outcome for children is better than for adults, with an 80% cure rate. Eighty percent of adults achieve complete remission, with 30% to 50% being cured. Without treatment, the life expectancy is about 3 months. |
| Complications: |
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| Calling your health care provider: |
| Call for an appointment with your health care provider if symptoms suggestive of ALL develop. Call your health care provider if persistent fever or other signs of infection occur in a person with ALL. |
| Prevention: |
| Because the cause of most cases is unknown, prevention of most cases is not possible. Minimizing exposure to toxins, radiation, chemicals, etc. may reduce risk. |
Reviewed By: Alan Greene, M.D., F.A.A.P., Chief Medical Officer, A.D.A.M.; Clinical Assistant Professor, Department of Pediatrics, Stanford University School of Medicine; Attending Physician, Packard Children's Hospital at Stanford (3/3/2002). Previous review by Tracy S. d'Entremont, M.D., Division of Hematology-Oncology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network (7/31/2001).
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