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| The different types of Beta Thalassemia |
One-gene and two-gene Beta Thalassemia |
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As explained in the introduction, Beta Thalessemia is caused by a definciency in Hemoglobin prduction in the Beta 'globin' chains, on chromosome 11. There are two major different types of Beta Thalassemia: One-gene thalassemia and two-gene thalassemia. (Alough different degrees of thalassemia are known, such as "Intermedia", such degrees are only found in about 10% of the cases and cause symptons that range in severity.) One-gene thalassemia is when one of the two beta-globin chains is normal, while the other is lacking in the abilily to produce sufficient hemoglobin, or unable to produce any hemoglobin at all. These people are known to be heterozygous and, in most cases, people who are afflicted with this one-gene form of the disease generally have no symptoms because the 'normal' gene is enough to function adequitly. People who are effected with the more severe type of Beta Thalassemia are not so lucky. The two-gene, or homozygous, form of this disease produces severe anemia on the Red Blood Cells and there is a severe deficiency in the beta chain production of hemoglobin. Consequently, people afflicted with this more severe type of beta thalassemia often need blood transfusions to survive. In addition, deformities are also common in people who have this two-gene form of the disease.
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Note that this two-gene form of the disease is also referred to as Cooley's anemia, Mediterrranean anemia, or von Jaksch's disease. |
