Alpha-thalassemia in Thailand.

The alpha-thalassemia syndromes are remarkable both for their phenotypic diversity and for their different clinical severity. They are associated with variable degrees of alpha-chain deficits; the clinical manifestations range from asymptomatic cases with normal hematologic findings to the totally lethal Hb Bart's hydrops fetalis syndrome. Recent molecular biology studies have clarified the defects in these alpha-thalassemia syndromes around the world. This paper describes the alpha-thalassemias in Thailand, and covers the types, molecular defects, incidence of each genotype, and their phenotypic expression.