Red Cell Indices and Hemoglobin Profile of Newborn Babies with Both the Sickle Gene and Alpha Thalassaemia in Central India.

Abstract

This study evaluated the effect of alpha thalassemia on the red cell indices and hemoglobin profiles of normal, sickle heterozygous and sickle homozygous newborn babies in central India where the sickle gene is linked to the Arab-Indian haplotype. 265 newborn babies were analysed with complete blood count and hemoglobin analysis on high performance liquid chromatography (Variant Hb Testing System, BioRad Laboratories, Hercules, CA, USA) using the β-thal short program. The sickle genotypes was confirmed by DNA analysis. The two common alpha gene deletions (- α3.7 and - α4.2) were detected by multiplex PCR. Among the 102 normal, 106 sickle heterozygous and 57 sickle homozygous newborns, the prevalence of a single alpha gene deletion (- α/αα) was 28.3% and that of deletion of 2 alpha genes (- α/- α) was 21.5%. In all, 57 normal (55.9%), 35 (33.0%) sickle heterozygous and 41 (71.9%) sickle homozygous newborns had a normal α genotype while - α/- α was seen in 23 (22.5%) normal, 30 (28.3%) sickle heterozygous and 4 (7.0%) sickle homozygous newborns respectively. The presence of associated alpha thalassemia resulted in a reduction in the hemoglobin levels and red cell indices in normal, sickle heterozygous and sickle homozygous newborn babies, MCV and MCH being strong discriminators of alpha thalassemia with two alpha gene deletions in all the three groups. This study also helped us to know the variations in hematological parameters in normal, sickle heterozygous and sickle homozygous newborns with and without associated α thalassemia.