Sickle cell disorder: A clinical and hematological outline of pediatric patients at a tertiary care teaching hospital in eastern India

Sickle cell anemia resulting from homozygosity for hemoglobin S (β^sβ^s) is a serious condition while heterozygosity for hemoglobin S (ββ^s), referred to as sickle cell trait, is usually asymptomatic. Sickle cell hemoglobinopathy is the best known hereditary blood disorder frequently seen in rural tribal population of eastern India with a prevalence as high as 30%. It is a genetically transmitted hemoglobinopathy responsible for considerable morbidity and mortality. This study was therefore undertaken to evaluate the clinical profile and correlate it with the hematological parameters of patients with sickle cell disease in a tertiary care hospital. This is a prospective observational study analysed from January 2018 to May 2019. All patients diagnosed as sickle cell disease and sickle cell trait were taken in this study. Other hemoglobinopathies associated with Hemoglobin S were not taken for analysis. A total of 61 patients in a period of approximately one and half year were evaluated, of which forty seven had sickle cell disease and 14 had sickle cell trait. Morbidity events were commonly observed in 5-12 years of age groups (68.85%). Seasonal variation was also observed, 47.54% of total cases were seen in winter season. Pain (60.65%) was the most common presenting symptom. Severe pallor (39.34%) and splenomegaly (24.59%) was the most common sign in both groups. Vaso-occlusive crisis (59.01%) was the most common morbidity event observed, of which abdominal pain was the most common site of pain. On statistical analysis, there was significant difference seen in illness and characteristics in both the groups. In patients with sickle cell disease acute painful crisis (59.57%) attributed to vaso-occlusion was very frequent even without the presence of precipitating factors while in sickle cell trait patients, acute painful episodes was associated with acute febrile illness (71.42%).