α-Thaiassemia in Thailand

Abstract

The α-thalassemia syndromes are remarkable both for their phenotypic diversity and for their different clinical severity. They are associated with variable degrees of α-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 α-thalassemia syndromes around the world. This paper describes the α-thalassemias in Thailand, and covers the types, molecular defects, incidence of each genotype, and their phenotypic expression.