Cooley s Anemia
- This is a preview of the essay.
To view the full text you must login!
Cooley's Anemia is a genetic disease that results when there is a defect in the synthese of one (or more) of the subunits of hemoglobin. The subunits are referred to as alpha or beta globin chains. As a result of a genetic defect red blood cells are small and hypochromic. Anemis is common. Incidence is females of Mediterranean descent for the purpose of identifying carriers of this potentially serious genetic disease.
Cooley's anemia (beta-thalassemia major, hereafter referred to as thalassemia) is a severe, inherited blood disorder characterized by a quantitative defect in the synthesis of the beta chain of hemoglobin caused by any one of more than 100 known mutations in and around the beta globin gene cluster. The disease is characterized by severe anemia beginning in the first six to twelve months of life. If untreated, the life expectancy is less than five years of age. Chronic red blood cell transfusions to maintain hemoglobin levels between nine and 11 gm/dl ("hypertransfusions") alleviate the anemia and partially suppress erythropoiesis. The regular administration of red blood cells also improves growth, delays or prevents enlargment of the liver, and spleen, and prevents the development of bone abnormalities that cause fractures as well as disfiguring changes known as Cooley's facies...