Increased Hb A level is a useful marker for differentiation of homozygous Hb E with and without α -thalassemia. This should lead to a significant reduction in number of referral cases of homozygous Hb E for molecular testing of α -thalassemia in routine practice.

This study emphasizes homozygous Constant Spring and compound heterozygous CS and Pakse as a cause of hydrops fetalis. Proper management for the fetus after diagnosis can lead to a good fetal outcome. Prevention control programs should include screening of parents for the heterozygous state.

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