Publication:

Hematological profile of sickle cell disease in Chhattisgarh

Background Sickle cell disease hemoglobinopathy gets inherited in autosomal recessive pattern. In sickle cell disease substitution of amino acid valine for glutamic acid at the sixth position on beta globin chain takes place resulting in sickled hemoglobin which is a hemoglobin tetramer.

Methods A prospective observational study was conducted in the Sickle Cell Institute, Raipur, India, and Department of Pharmacology in collaboration with Department of Biochemistry, Pt. J.N.M. Medical College, Raipur, Chhattisgarh, India, from February 2018 to June 2018. Patients included were in the steady state for a long period of time without any symptoms related to sickle cell disease or any other diseases which could affect hematological parameters. Subjects transfused in the last three months were excluded. Student t test and Pearson Correlation Coefficient test was done on stat pages and socscistatistics calculators. P-value<0.05 was considered as statistically significant.

Results A total of 50 subjects of sickle cell disease homozygous (SS) were studied for hematological parameters. The mean age±SD of 50 subjects were 13.3±9.24 years. Out of 50 subjects, 35 were males and 15 were females. Total RBC count, mean corpuscular volume (MCV), mean corpuscular haemoglobin (MCH) and mean corpuscular hemoglobin concentration (MCHC) was low in present study. Significant inverse correlation was found in females between HbA2 and HbF, p=0.01, while it was insignificant and negatively correlated in males being 0.23.

Conclusions Sickle cell disease homozygous is a common and challenging health problem of Chhattisgarh population.