BACKGROUND Sickle cell anaemia is a commonly encountered inherited haematological disorder in Central India. Despite the unicity of the genetic mutation that is responsible, clinical presentation of the sickle cell haemoglobinopathy was found to be different according to regions and patients. Aims and Objectives-To study the clinical profile of sickle cell anaemia with particular reference to vaso-occlusive crisis and to estimate the quantity of foetal haemoglobin (HbF) with High Performance

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... uid Chromatography (HPLC). MATERIALS AND METHODS In this descriptive study, 120 cases of sickle cell anaemia (SS pattern), having age 12 years and above, attending medicine and/or sickle cell OPD or admitted in medicine indoor wards were included. Cases were subjected to detailed history, clinical examination and laboratory investigations including HPLC. Various parameters (clinical and laboratory) and quantity of HbF were studied. RESULTS Maximum cases, 70 out of 120 (58.3%) were in 13 -19 age group range. Mean age of onset of symptom was 8.04 years. Vasoocclusive crisis (VOC) was the most common manifestation followed by severe anaemia, while leg ulcers and stroke were rare presentations. The frequency of VOC varied widely among cases with range of 0.3-3 episodes per year, the average frequency being 0.6 episodes per case per year. The most common site of pain in VOC were limbs (68.7%), hands (62.5%), knees (59.3%), abdomen (31.2%) and chest (25%). Fever was the most common precipitating factor for VOC (31.2%). The mean HbF level was 21.9%. CONCLUSION Vaso-occlusive crisis was observed to be the most common presentation in sickle cell anaemia. Sickle cell anaemia patients had high levels of foetal haemoglobin from this region.