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Wednesday 15 June 2011

Acute Lymphoblastic Leukemia

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Acute Lymphoblastic Leukemia


Acute lymphoblastic leukemia(ALL) is a disease that occurs in the blood-making system of the body. ALL begins when the red blood cells are overcrowded by the white blood cells. This overproduction of white blood cells can cause Immune Hemolytic Anemia. Immune Hemolytic Anemia is a stronger and more destructive disease that affects the body’s immune system by prematurely destroying red blood cells and weakening the body’s infection fighting abilities. If Acute Lymphoblastic Leukemia is found early enough it can be cured and sent into remission in almost all children and in many adults.


Causes and Incidence


Acute Lymphoblastic Leukemia results from a few different genetic factors. The genetic defect known as Ikaros may be responsible for a great deal of the ALL cases in infants. This defect causes an abnormality in the lymphocyte development. The genetic defect known as the Philadelphia (Ph) chromosome is also responsible for about 0 percent of the ALL cases in adults and 5 percent in children. There have also been cases of ALL caused by a genetic defect called TEL-AML1. This defect is easily treated with chemotherapy. The largest contributor to Acute Lymphoblastic Leukemia and all other Leukemia’s is genetic rearrangement, called translocation. Translocation is responsible for 65 percent of Leukemia cases. This is when some of the genes are changed or shuffled between a pair of chromosomes. 0 percent of ALL patients have a translocation of the 1 and 1 chromosomes which is the most least threatening case. Younger patients who have translocations in the 4 and 11 chromosomes and the and chromosomes have a very severe case.


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Signs and Symptoms


Acute lymphoblastic leukemia can be very difficult to recognize because its early signs are similar to those of the flu. Things like persistent fever, frequent infection, bone and joint pain, weakness, and swollen lymph nodes are all common to ALL. In children symptoms are also things like changes in energy and appetite, short attention span, and irritability. All of these symptoms often happen abruptly and intensely, they can also be present one day and then appear to be gone the next. These symptoms begin because there is not enough healthy mature leukocytes to allow the immune system to work properly. There are not enough healthy platelets to stop bleeding and the amount of red blood cells is extremely low causing the body to be low in oxygen. Anemia begins to develop, the body begins to have poor color and bruise easily. Uncontrolled bleeding and even getting extremely tired easily are also good signs of ALL. Thrombocytopenia is another symptom; this is when small red dots begin to appear on the skin.


Diagnosis


Diagnosing Acute Lymphoblastic Leukemia is extremely difficult and it is not uncommon for it to go misdiagnosed for a long time. ALL has symptoms very similar to the flu and people wait sometime before even going in for a intensive diagnostic. ALL also has very similar symptoms to diseases like acute myelocytic leukemia, hairy-cell leukemia, and malignant lymphoma which makes it difficult to make a proper diagnosis. When a child is found to have disease in the bone he or she may be misdiagnosed with juvenile rheumatoid arthritis or osteomyelitis. There are three steps that the patient goes through in order to be diagnosed with Acute Lymphoblastic Leukemia. First the doctors complete a complete blood cell count. This test will show the amount of both red and white blood cells. A patient with ALL may have test results showing either high amounts of white blood cells or low levels of red blood cells. There is not always a presence of leukemia cells and sometimes the results are perfectly normal even though the patient does have ALL. The next step in the diagnosing process is a bone marrow biopsy. The doctor inserts a needle in the patient’s hip or sternum bone and removes a bone marrow sample. This sample is examined under a microscope for signs of uncontrolled cell growth. A normal or healthy patients bone marrow contains 5 percent of growing cells, but a leukemia patients bone marrow contains anywhere from 0 to 100 percent of growing cells. The final step before giving the diagnosis is complete is testing for certain physical characteristics of the cells or morphology. The bone marrow sample is combined with staining solutions to dye certain parts of the cell. The cell line is examined to differentiate myeloid or lymphoblastic leukemia. Then if the levels of terminal deoxynucleotidyl transferase (TdT) are elevated it usually is a positive sign of acute lymphoblastic leukemia. At this point if ALL is found to be present then a few more genetic tests are conducted to determine the severity of the case and the treatment method. Also at this point if ALL is confirmed doctors would perform a spinal tap which removes a sample of cerebrospinal fluid to determine if the leukemia has spread to the central nervous system.


Treatment


Treatment for acute lymphoblastic leukemia takes place in four stages, induction therapy, central nervous system (CNS) prophylaxis, consolidation, and maintenance. The goal of treatment is to send the ALL into remission or where there are no signs of leukemia in the body and the bones have a 5 percent or lower level of growing cells. Chemotherapy or radiation is the main form of treatment in each stage of the treatment. The different stages just determine the intensity of the therapy, the amount of drugs administered, and the focus of the therapy. The Induction Phase is the most intense and is the hardest on the body. Here the objective is to rid the body completely of leukemia cells in order to prevent spreading to the brain or spine. Hospitalization is necessary for some of the blood-administered drugs. Usually remission is achieved during this phase of therapy. Only severe patients and those patients over the age of 60 are not in remission after this stage. The next stage is called central nervous system (CNS) prophylaxis and is for the more serious cases. At the point doctors are focusing more on sanctuary disease areas like the brain, spine, and testes. Chemotherapy is again administered in different amounts to these areas in order to remove and prevent leukemia cells from spreading. All patients go through the next stage called consolidation therapy. This is simply smaller doses of radiation to the entire body in order to remove any lingering leukemia cells. This goes on for about six months after remission is achieved and it is simply a preventative measure to make sure the leukemia does not relapse. The last stage, maintenance, is similar to the consolidation stage where the patient is simply attempting to prevent the leukemia from relapsing. The patient takes medication and has fewer weaker doses of radiation therapy. There is no exact length to this stage because there is no definite cure for leukemia. However, children usually continue the maintenance stage for to years after remission. Adults maintain themselves a little longer than children, but they also have to keep an eye on their blood count by taking tests on a given schedule.





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