Variant sickle cell syndromes Sickle cell trait

Sickle cell trait (HbAS) is a benign condition that has no haema-tological manifestations and is associated with normal growth and life expectancy. Sickle cell trait affects 8-10% of African-Americans and up to 25-30% of the population in West Africa. Upon electrophoresis, the ratio of HbA-HbS is 60:40, owing to the greater affinity of a-globin chains for PA-globin chains. Impaired urine-concentrating ability and haematuria can occur, and an increased incidence of urinary tract infection is observed in pregnant women with sickle cell trait. A slight risk of sudden death during military training has been reported, and splenic infarction is possible at very high altitudes. Genetic counselling should be provided to individuals with sickle cell trait.

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