Abstract
The α-thalassemias are the most common inherited disorders of hemoglobin (Hb) synthesis due to deletions or point mutations. Alpha globin is made by four genes, two on each strand of chromosome 16. Individuals who have one or two abnormal alpha globin genes have alpha thalassemia trait. It is commonly found in Africa, the Middle East, India, Southeast Asia, southern China, and occasionally the Mediterranean region. When a person has only one functional alpha globin gene, they have Haemoglobin H disease, require regular medical care and may experience lifelong anaemia of mild to moderate degree. When a person has no alpha globin genes, they have a severe condition called Hb Bart's hydrops fetalis. It affects a foetus long before birth, resulting in death during pregnancy or shortly after birth. Those with Haemoglobin H disease may require blood transfusions to correct anaemia. There is no treatment or cure for Hb Bart's hydrops fetalis.
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