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

Abstract

Background: Sickle cell syndromes are commonly encountered inherited haematological disorders regarding which sparse published data are available from Telangana State. Methods: Prospective study of 55 patients diagnosed to have sickle cell syndromes at our tertiary care teaching hospital in Hyderbad, Telangana State, 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 %). Conclusions: Sickle cell disease should be considered in the differential diagnosis while evaluating patients presenting with anaemia and skeletal pains. Prompt recognition and management improves survival and eventual prognosis in these patients.