See also Thalassemias

Most common hemoglobinopathy after the sickle cell disease. More prevalent in people originating from South-East Asia, where it is often associated to a thalassemia. It seems to protect against paludism. It is an abnormal hemoglobin resulting from the point mutation ß26 [B8] Glu SLys. This results in a defect in the synthesis of one of the β chain of hemoglobin. 

Clinical pictures:

-        heterozygous A/E: asymptomatic

-        homozygous: E/E: moderate hemolytic anemia

-        heterozygous E / β°: clinical picture of intermediary β-thalassemia 

-        heterozygous S/E: severe sickle cell syndrome

Anesthetic implications: 

complete blood count, bilirubin level

References : 

Updated: October 2021