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Sickle Cell Disorder Frequency: Hb S / Thalassemia

Hb S / thalassemia combinationAffected populationsSeverityComments
Hb S beta thalassemia
North Africa, India, and the Mediterranean region, especially Greece and Turkey.
Varies
Hb S beta-plus thalassemia, type 1 and Hb S beta-minus thalassemia need supportive therapy and may have severe anemia
Hb S beta-plus thalassemia, type 2 requires very little medical attention
Hb SA alpha-plus thalassemia
Common in persons of African ancestry
Usually asymptomatic
Less hemoglobin S produced than in persons with Hb S trait
Hb SS-alpha thalassemia (either plus or zero)African and Mediterranean ancestryMild anemia midway in severity between sickle cell disease and trait
Produce increased levels of Hb F in proportion to the number of alpha gene deletions present. This acts to retard the sickling process.