ALPHA-THALASSEMIA AND BETA-THALASSEMIA AMONG CHINESE CHILDREN IN GUANGXI PROVINCE, PR CHINA - MOLECULAR AND HEMATOLOGICAL CHARACTERIZATION

We have studied nearly 100 patients with beta-thalassaemia major and 6 0 patients with Hb H disease who were attending the Haematology Clinic of Guangxi Medical College, Treatment of the patients was Limited and only a few patients with beta-thalassaemia major received blood trans fusion(s). As a result, the severe anaemia has led to early death at 3 -4 years for beta(degrees)-thalassaemia homozygotes, and 8-12 years fo r beta(+)-thalassaemia homozygotes. Four beta-thalassaemia alleles are responsible for nearly 90% of all beta-thalassaemia chromosomes. This information has resulted in the initiation of a prenatal testing prog ramme at the local level. The patients with Hb H disease maintained a haemoglobin level of 6-10 g/dl and early death was infrequently observ ed. The --(SEA) deletion was the major type of alpha-thalassemia-l, wh ile three smaller deletions (-2.7, -3.7 and -4.2 kb) and two nondeleti onal alpha-thalassaemia determinants (Hbs Constant Spring and Quong Sz e) were the alpha-thalassaemia-2 types.