This syndrome is observed in locations in which both Hb S and P-thalassemia are frequent, such as Africa, Sic ily, Greece, Turkey, the Arab countries and the regions of America with African and southern Mediterranean admixture.
The genotype may be S/p+ thalassemia, in which the red cells contain between 20 and 40% Hb A, the remainder comprising Hb S and Hb F; or S/p0 thalassemia, in which the red cells contain only Hb S and Hb F. The latter genotype can only be diagnosed by pedigree or genetic analysis.
The clinical picture of S/p0 thalassemia is very similar to that of sickle cell anemia but it is milder. Anemia may be milder than Hb SS, and in some cases the mean cell volume (MCV) is lower. Retinopathy and osteonecrosis are more common, but autosplenectomy is less common.
The clinical picture of S/p+ thalassemia is significantly milder than that of sickle cell anemia: there is less anemia, a lower MCV and a lower reticulocyte count but an increased risk of retinopathy and osteonecrosis. The spleen often enlarges during adulthood.
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