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LEUKEMIA
About this Cancer
 
Leukemiais a disease of the white blood cells. All types of blood cells are produced by the bonemarrow (the spongy tissue inside the large bones of the body): red blood cells, which carry oxygenand other materials to all tissues of the body; platelets, which help make the blood clot; and whiteblood cells, which fight infection.All of these cells arise from stem cells, which are blood cells at their very earliest stage of development. Stem cells can develop into different types of white cells, including lymphocytes andneutrophils.
 
In leukemia, the immature white cell loses its ability to mature and specialize (differentiate) itsfunction. As a result, the marrow produces too many of one type of cell that replaces normal bloodcells and results in bone marrow failure. These abnormal white cells flood the blood stream andlymph system, and may invade vital organs such as the brain, testes, ovaries, or skin.The bone marrow is the factory for all new blood and immune cells. When it does not work correctly,cells that prevent bleeding and infection, or that carry nourishment and oxygen to vital organs andtissues, cannot do their jobs properly.
 
Leukemia can be acute, progressing quickly with many immature cancer cells, or chronic, progressingslowly with more mature-looking cancer cells.
 
The most common type of leukemia in children is acute lymphocytic leukemia(ALL). Other types of leukemia that occur less frequently in children are acute myeloid leukemia(AML), and chronicmyeloid leukemia(CML).
Acute Lymphocytic Leukemia (ALL):
 
Acute lymphocytic or lymphoblastic leukemia, known as ALL, is the most common childhoodleukemia, accounting for 80 percent of all acute leukemias in children. ALL is most common inchildren between the ages of 3 and 7. It occurs when too many immature lymphocytic cells(lymphoblasts) are produced and multiply rapidly, crowding out normal blood cells and making thechild susceptible to bleeding and infection. These cells can be found in the blood, the bone marrow,the lymph nodes, the spleen, and other organs.There are two different types of lymphocytes: T cells and B cells. Often the leukemia arises in theimmature lymphocytes. Less frequently, however, leukemia cells develop in T or B cells beforebecoming cancerous. This type of lymphocytic leukemia is referred to as T or B cell leukemia.
 
Risk Factors
 
Most cases of ALL seem to have no apparent cause. However, radiation, some toxins such as
 
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benzene, and some chemotherapy agents are thought to contribute to the start of leukemia.Abnormalities in chromosomes may also play a role in the development of acute lymphocyticleukemia.Risk factors for ALL include Down syndrome, a sibling with leukemia, and exposure to radiation,chemicals, and drugs. The incident rate is six out of 100,000 children.
 
Cancer Symptoms
 
As the leukemia cells multiply in the bone marrow, the production of normal blood cells slows, so thechild may become tired and lethargic, or short of breath due to anemia caused by a lack of oxygencarrying red blood cells. Bruises may develop, and bleeding may occur due to low numbers of clottingcells called platelets. Sometimes, a child may suffer from infections because of low numbers of normal white blood cells.
 
A child is likely to feel unwell in general and may complain of aches and pains in his or her arms, legs,or joints, and/or have swollen gums or glands. Other signs and symptoms may include nosebleeds,excessive bruising, pinpoint red spots on the skin (petechiae), fever, weight loss, and the sensation of an irregular heart rate (palpitations). Often symptoms can mimic those of a viral infection, but whenthey continue for more than a week or two, other causes should be investigated.
 
Diagnostic Tests
 
Blood tests are administered to determine if there are abnormal leukemia cells in the blood stream,and if the number of normal white blood cells, red blood cells and platelets is low. A sample of bonemarrow is removed from the hip bone (biopsy) to confirm the presence of abnormal white blood cells(leukemia cells) in the bone marrow. A lumbar puncture (spinal tap) is done to remove a sample of spinal fluid to see whether it contains any leukemia cells. A chest X-ray will indicate any enlargedglands in the chest.
 
Other tests may be necessary, depending on the child's symptoms.
 
Classification of ALL currently depends on a number of specific, sophisticated tests, such asimmunophenotyping, karyotyping, and terminal deoxynucleotidyltransferase (TdT) activity. Thecombined results of these tests allow pinpoint molecular diagnosis, which helps guide the treatmentdecisions, and clarify the likely prognosis.
 
For example, the cells of some leukemias contain chromosomal abnormalities. Those with thePhiladelphia chromosome, or with the t(4;11) translocation, have a poorer prognosis, and intensivetreatment, including an early bone marrow transplant, might be recommended. Other genes canindicate a very favorable prognosis.
 
Treatment
 
Chemotherapy (oral and/or intravenous treatment with anticancer drugs)
 
is the main treatment forALL and has several phases:
 
Induction:
This phase lasts for approximately four weeks, and typically requires the child to stay inthe hospital. A bone marrow test is taken at the end of the induction treatment to see if the leukemiais in remission, meaning there is no evidence of leukemia.
 
Intensification therapy:
Even if bone marrow tests do not show leukemia cells, indicating the
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patient is in remission, it is possible that some cells have survived. Additional chemotherapy drugsare given to destroy any of these remaining leukemia cells that may have survived the inductionphase of treatment. These are usually given in three blocks of intensive treatment.
 
Central Nervous System (CNS) treatment:
ALL sometimes develops in the brain and spinal cord.Doctors try to prevent this by injecting a drug, usually methotrexate, directly into the spinal fluid.Occasionally, radiation therapy (targeted X-rays to destroy tumors) to the brain is also necessary.
 
Maintenance or consolidation therapy:
This phase of chemotherapy treatment lasts for up to 21/2 years from diagnosis and involves taking daily tablets at home and having intermitent,intravenous chemotherapy (treatment with anticancer drugs). During maintenance treatment mostchildren will be able to take part in their normal daily activities.
 
Bone marrow/stem cell transplantation (BMT):
Since leukemia is a disease caused by abnormalbone marrow function, bone marrow or stem cell transplantation (BMT) can potentially cure thedisease when chemotherapy fails. In this treatment, the diseased bone marrow is destroyed usinghigh doses of chemotherapy (anticancer drugs) and radiation. Healthy donor (allogeneic) stem cellsor bone marrow, or self-donated (autologous) stem cells or bone marrow, purged of any leukemiacells, are given to the patient intravenously and travel to the bone to repopulate the bone marrow.This treatment is only indicated in cases where the ALL is likely to recur following standardchemotherapy, or for children whose leukemia has come back following standard treatment.
 
During treatment, some patients may require the transfusion of blood products to treat anemiaand/or bleeding.
 
Acute Myeloid Leukemia (AML):
 
Acute myeloid leukemia, known as AML, occurs when too many neutrophils develop from immaturecells of the myeloid line. As the production of these abnormal cells increases, production of normalcells decline. AML is less common in children than ALL.
 
Risk Factors:
 
In general, the causes of AML are unknown. Children with Down syndrome have an increased risk of developing AML during the first three years of life.
 
Cancer Symptoms
 
As the leukemia cells multiply in the bone marrow, the production of normal blood cells slows, so thechild may become tired and lethargic, or short of breath due to anemia caused by a lack of oxygencarrying red blood cells. Bruises may develop, and bleeding may occur due to low numbers of clottingcells called platelets. Sometimes, the child may suffer from infections because of low numbers of normal white blood cells. A child is likely to feel unwell in general, experience fever or chills, and maycomplain of aches and pains in his or her arms, legs, or joints.
 
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