Death due to sickle cell anaemia, an autopsy diagnosis: a study at a tertiary care hospital

Sickle cell anemia was first described by Herrick in 1910. Pioneering studies by Pauling et al. established that Sickle Cell Disease (SCD) results from a defect in the hemoglobin molecule. 1 The sickle mutation was characterized several years later by Ingram et al. as a glutamine-to valine substitution at the sixth residue of the beta globin polypeptide. Homozygosity for the sickle mutation (i.e., HbSS) is responsible for the most common and most severe variant of SCD. Sickle cell disease is an autosomal recessive, genetically transmitted haemoglobinopathy which is responsible for a considerable amount of morbidity and mortality. 2