The haematological, biochemical and clinical--presentation of haemoglobin S in Saudi Arabia (i). Haematological & clinical expression.

The haematological and clinical expression of haemoglobin S (Hb S) was investigated in a group of male and female Saudis of haemoglobin genotype AS and SS, and the results compared with those from normal individuals (AA). The influence of alpha thalassaemia in each genotype was also investigated. The total haemoglobin concentration (Hb), red blood cell count (RBC) and packed cell volume (PCV) were significantly lower in the SS group without thalassaemia. In the thalassaemic group Hb, RBC and PCV levels were higher, while MCV and MCH were considerably lower. The clinical investigations reveal that almost 30% of the SS cases had not required blood transfusion, and none of the patients had suffered from leg ulceration or hand and foot syndrome. Comparison of the results obtained for Saudi sickle cell patients with those reported for Jamaicans show several differences, indicating a milder disease in the Saudis.