Hemoglobinopathy S

Hemoglobinopathy S is an entity that includes all congenital hemolytic anemias in which the gene coding for Hb S is inherited. Worldwide there are an estimated 300 million people recognized as hemoglobin S carriers. There are an estimated 300 million people in the world recognized as carriers of hemoglobin S (Hb S), a condition resulting from the substitution of glutamic acid for valine at position 6 of the ß-globin gene on the short arm of chromosome 11, which are characterized by the inheritance of a normal hemoglobin A gene from one parent and an abnormal mutated gene from the other parent (AS), unlike homozygous individuals in which both mutated genes are present (SS).