This paper reports the case of a 17-year-old male student from the Jaizan area in south-western Saudi Arabia who had sickle cell anaemia and possessed three α-genes on one chromosome (αααanti3.7) and two on the other. The clinical manifestations were severe, with frequent blood transfusion requirements and frequent episodes of painful crises, severe anaemia and tissue involvement. In comparison with age and sex-matched sickle cell anaemia patients with one α-gene deletion (-α/αα), or a normal α-gene arrangement (αα/αα), a more severe disease presentation was obvious in the propositus. It is suggested that with the surplus α-globin chains, more severe haematological and clinical abnormalities occur, these influence the phenotypic expression of sickle cell anaemia. However, more patients with this type of gene arrangement must be studied before a definite conclusion can be reached regarding the influence of excess α-globin chains on the presentation of sickle cell anaemia.

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