Leukemia

Acute Lymphoblastic Leukemia

 Acute lymphoblastic leukemia, also known as ALL, is the most common form of leukemia that occurs in children. It is characterized by the presence of too many immature white blood cells in the child’s blood and bone marrow. While ALL can occur in adults too, treatment is different for children.The term “acute” refers to the tendency of this disease to progress rapidly. “Lymphoblastic” refers to the white blood cells, which are also called lymphocytes. Normally, lymphocytes mature and become an important part of the body’s defense system against infections.

In ALL, something happens — researchers do not yet know what, but something interrupts normal cell development. The result is an overabundance of incompetent, “blastic” immature cells. Their presence affects a person in two ways:

1. By crowding. Lymphocytes are made in the bone marrow, the spongy tissue inside the large bones of the body. But other vital blood components are made there, too — red blood cells needed to carry oxygen to tissues, and platelets that are needed to stop bleeding through clotting. When immature lymphocytes crowd out red blood cell production, a child’s body does not receive all the oxygen it needs. As a result, he or she may develop anemia. And when immature lymphocytes crowd out platelets, the child bleeds and bruises easily.

2. By invading other organs, such as the spinal cord and brain, liver, or spleen. The presence of lymphocytes in these sites impairs function here, too.

To describe the scope of most cancers, doctors have devised a "staging" system. In it, stage 1 cancer is a cancer that has not spread. Stage 4 is an advanced cancer that has spread to multiple sites. ALL is different in this respect as the bone marrow is present throughout the body. There is no staging system. Because of Claire's age at original diagnosis (15), she is considered high risk. Claire also has an abnormality within the leukemia cells. She has the Philadelphia chromosome, which is pretty rare, occurring in only 2-10% of ALL cases. Normally all individuals have 46 chromosomes in their cells, arranged in 23 pairs. 1 is the shortest and 22 is the longest. In the leukemia cells chromosome number 9 and number 22 break and fuse (translocation). This fusion product is called bcr-abl. Each part represents the place (the gene) where the break happened in the 2 chromosomes. The drug Gleevec and it's next generation drugs specfically targets this abnormality.

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