PHENOTYPIC-EXPRESSION OF HEMOGLOBIN A(2) IN BETA-THALASSEMIA TRAIT WITH IRON-DEFICIENCY

Iron status was estimated in 463 heterozygous beta-thalassemics to del ineate the effect of iron deficiency on the expression of hemoglobin A (2) (HbA(2)) in these patients. One hundred and twenty-six (27.2%) pat ients with the trait were iron deficient. These iron-deficient patient s had a significantly (p < 0.0002) higher prevalence of anemia (90.5%) compared with iron-replete patients with the trait (71.5%). The mean hemoglobin (Hb) concentration, MCV, and MCH were significantly (p<0.00 01) lower in patients with beta-thalassemia traits (BTT) who had iron deficiency than in those without iron deficiency. Mean RBC count and M CHC did not differ in the two groups. Mean HbA2 was not significantly different in the two groups of patients with the trait and was elevate d (>3.5%) in all but one heterozygote investigated. Mean HbA(2)/cell w as significantly (p < 0.05) lower in BTT patients with iron deficiency than in patients without iron deficiency. The presence of iron defici ency did not preclude the detection of BTT in this population. The eff ect of iron deficiency in BTT was apparent as a significant lowering o f the Hb concentration and an increased prevalence of anemia. Iron the rapy is warranted for BTT patients with iron-deficiency traits and wou ld help to significantly raise their Hb concentration. The elevation o f HbA(2) was striking and could be used with reliability in making the diagnosis of BTT even in the presence of iron deficiency.