Sickle cell disease: Experience of a tertiary care center in a nonendemic area

Abstract

Sickle cell disease is an genetically transmitted hemo-globinopathy. It is prevalent in many parts of India, where the prevalence has ranged from 9.4-22.2% in endemic areas. There is paucity of data with respect to its prevalence and manifestations in the non endemic areas of India. The present study attempts to evaluate varied manifestations of sickle cell disease in a tertiary care center in north India, which is a non-endemic belt. It was observed that 18% of the patients in the study to be the local inhabitants of Delhi- a nonendemic belt. Also this region had the largest percentage of the mixed cases particularly associated with beta thallesemia. A high index of suspicion is therefore required for the diagnosis of such cases in a nonendemic area like Delhi specifically in patients who presented with unexplained anemia and splenomegaly with or without pain abdomen. Various crises reported in sickle cell disease is not a common manifestation especially in a nonendemic belt in our experience.