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What is alpha thalassemia?
2/26/2013 11:02:52 AM
There are at least five main types of alpha thalassemia. These are most common in people of Southeast Asian, Indian, southern Chinese, Middle Eastern and African ancestry (4). There are four genes that control the production of alpha globin. The severity of the condition is determined by how many of these genes are missing or abnormal.
- Silent carrier, the mildest form, has one alpha globin gene missing or abnormal. Affected individuals generally have no symptoms, but they can pass on the genetic abnormality to their children.
- Alpha thalassemia minor (also called alpha thalassemia trait) has two missing or abnormal alpha globin genes. Affected individuals may have no symptoms or a mild anemia, but they can pass the condition on to their children.
- Hemoglobin H disease is caused by three missing or abnormal alpha globin genes (so there is one normal alpha globin gene). The condition causes abnormalities in red blood cells and rapid destruction of these cells. Most affected individuals have mild to moderate anemia and can live fairly normal lives. The anemia may temporarily worsen when individuals have a viral infection or when they are treated with certain medications (such as sulfa drugs) (5).
Some affected individuals eventually develop complications, such as an enlarged spleen or gallstones (5). Individuals with hemoglobin H disease should receive regular medical care to detect and treat these complications. Some may need occasional blood transfusions (6).
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