Clinical profile of sickle cell syndromes: experience at a tertiary care centre in South India

Results: Their mean age was 19.9 (range 3-48) years; there were 35 males. Consanguinity was noted in 31%. History of cholecystectomy was evident in 5 cases. Blood transfusions were received in the past in 52% of cases. Symptoms at presentation were jaundice (85%), pain (80%), fatiguability (60%), pallor (30%), dyspnoea (29%), lump abdomen (7%) and leg ulcer (3%). Acute chest syndrome was seen in 10.9% cases. Physical examination revealed pallor (90%), icterus (80%) hepatomegaly (49%) and splenomegaly (41%). Mean haemoglobin at presentation was 8.3 g/dL. Sickle cells were seen in peripheral smear in 51%. Sickling test was positive in all after induction. Characterization of haemoglobin by high performance liquid chromatography revealed homozygous sicke cell anaemia was evident in 22/ 43 (51.2%), sickle thalassemia in 16/43 (37.2 %) and sickle cell trait in 5/43 (11.6 %).