Interaction of hemoglobin E with other abnormal hemoglobins.

摘要

Hemoglobin E (Hb E) (HBB Glu26Lys) and hemoglobin S (Hb S) (HBB Glu6Val) are the most common hemoglobin variants in the world. Hb E trait and Hb EE are mild disorders and are clinically asymptomatic. However, the compound heterozygote state of Hb E-beta-thalasse-mia results in a heterogeneous phenotype ranging from transfusion dependence to a complete lack of symptoms. Hb E has been reported in a compound heterozygous state with other variant hemoglobins like Hb C (HBB Glu6Lys), Hb Malay (HBB Asnl9Ser), and Hb S. Hb S is prevalent in several parts of India, with frequencies reaching as high as 22% in Central India [1]. Hb E is common in the northeastern regions of the country, with frequencies of 4-50% [2, 3]. Hemoglobin D-Punjab (Hb D) (HBB Glul21Gln), an abnormal hemoglobin, is widespread in Northwestern India [4].