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Alpha Thalassemia Fact Sheet

Source: www.dhs.ca.gov
Topic: Thalassemia
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Sort Desciption: Thalassemias are a group of disorders which have in common the inability to produce sufficient quantities of globin chains necessary for hemoglobin synthesis. The most common types of thalassemias are ...

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Thalassemias are a group of disorders which have in common the inability to produce sufficient quantities of globin chains necessary for hemoglobin synthesis. The most common types of thalassemias are alpha and beta, which are named according to the type of chain affected. The focus of this material is alpha thalassemia. Alpha Thalassemia There are four genes which code for alpha chain production. Alpha thalassemia results when one or more of these genes are not working. The number and location of the non-working genes determines the type of alpha thalassemia an individual has. Types of Alpha Thalassemia Alpha thalassemia major (Hydrops Fetalis): Deletion of all four alpha globin genes. No alpha chains, which are necessary for the formation of fetal hemoglobin, are produced. Death usually occurs in utero or early infancy. Treatment consists of ongoing transfusions. Hemoglobin H (Hb H) disease: Deletion of three alpha globin genes. The clinical complications associated with Hb H disease are variable. This generally results in mild to moderate anemia, and is often associated with microcytosis, hypochromia, and red cell fragmentation. Hemoglobin H is an abnormal hemoglobin found in people with alpha thalassemia. When three or more alpha globin genes malfunction, there is an excess of beta globin chains. The excess chains create unstable tetramers called hemoglobin H. The tetramer of beta globin chains (4 ) forms when there are insufficient alpha ( ) chains to make normal adult hemoglobin (2, 2). The fetus manufactures gamma ( ) chains rather than chains, and the tetramer of chains that forms is called hemoglobin Barts ( 4). During the newborn period, when gamma globin production is still high and beta globin production is low, the gamma chains form the unstable tetramers identified as hemoglobin Barts. However, Hb Barts decreases with the normal decrease in gamma chain production and therefore, over ...

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