Two different patterns of sickle cell disease in children in Saudi Arabia

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Abstract
SUMMARY Forty-five Saudi children with sickle cell homozygote state were followed up for two years. Two different patterns of disease emerged. One, which was of mild nature and unusually high fetal haemoglobin (Hb F) level, occurred among children whose parents were from the Eastern Province of Saudi Arabia: it was similar in pattern to sickle cell disease already described in Arabs from that province and from neighbouring Kuwait and the Gulf area. The other type of disease, in which Hb F levels were low, was more severe, had a higher mortality and was seen among children whose parents originated from the south-western part of Saudi Arabia: the clinical picture and haematological findings in this type were similar to the disease pattern described in negroes. It is concluded that two different patterns of sickle cell disease dependent on Hb F levels occur in Saudi Arabia and not only the mild form which has been repeatedly described and emphasized (1, 2, 3, 4). Other genetic and environmental factors, not studied here, may also play a differentiating role.