Hematological status of β-thalassemics in Madras

Although rapid technical advances have taken place in the diagnosis of β-thalassemia, still the hematological factors were found to be suitable screening test in areas like Indian subcontinent where a high prevalence of β-thalassemia trait was observed. Among various thalassemias reported in Asian Indians, β-thalassemia account for about 80% and is responsible for very high infantile mortality. Despite this, little is known about the hematological status of β-thalassemias among this ethnic group which is associated with more than five different predominant β-globin mutation with high frequency and variable number of rare ones. The present study is the first report of hematological status of β-thalassemia among this ethnic group particularly from Tamil Nadu, Southern India, who are still practising high degree of consanguinity. In the present study, a total number of 364 β-thalassemics were investigated. This includes 84 cases of homozygous β-thalasemias and the remaining 280 were heterozygotes. The hematological factors such as red cell indices, hemoglobin F and hemoglobin A₂ were assessed. The results revealed a wide spectrum of hematological variables ranging from severe form as that of Mediterranean thalassemias to very mild form of anemia as that of african Negro population.