Gene Expression in Langerhans Cell Histiocytosis
Kenneth L. McClain MD, PhD
Baylor College of Medicine, Texas Children’s Cancer Center – Houston, Texas USA
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Better therapies for Langerhans cell Histiocytosis cannot be designed until we understand what causes the disease to occur. It is known that several genes that regulate the immune system are making their products at much higher levels than normal immune cells. However, the number of genes studied so far is rather limited, less than 20.
We have developed a method to isolate the individual immune system cells that are in the diseased tissues of patients with LCH and study the genetic information that is being used from 96 genes. Our methods allow us to measure the amount of each gene and compare that number to cells from different patients. The importance of this is that differences in the kinds and amounts of genes being used in LCH patients with only bone or skin lesions can be compared to those with liver, spleen, lung, or bone marrow LCH. The latter group of patients typically is harder to treat and only about half of them survive. By understanding the differences between these two groups of patients we will be able to concentrate on a few of the most important genes and learn why they are working in abnormal ways.
Another goal of the project is to identify patients early in their treatment who have too high a chance of not being cured by current treatments and giving them additional therapy early so they may have a better chance of survival.