Clinical Spectrum of Patients with Beta Thalassaemia: A Review of Fifty-Four Patients

Background: Beta thalassaemia is a common haematological disorder presenting as anaemia through out the world including Pakistan. Although nutritional anaemias and anaemias due to worm infestation are the leading cause of anaemia in our children but thalassaemia is also an important cause of increased morbidity and mortality in children belonging to this part of the world. This study was undertaken to know the clinical aspects of this disease. Material & Methods: This study was carried out in children Centre, Military Hospital, Rawalpindi during one year period from October 2000 to September 2001. A total of 54 cases of ß thalassaemia were included in the study. Out of these, eight patients were referred for the first time and were newly diagnosed while others were already diagnosed. In all these cases history was taken and detailed clinical examination carried out. Blood picture and haemoglobin level of the patients and parents were performed along with other necessary laboratory investigations. Results: Majority of these patients were from the rural areas of Rawalpindi, belonging to Rajput, Awan and Mughal families. Age range was from one month to three years. Male to female ratio was 2.4 : 1.0. All these patients were having severe anaemia and hepatosplenomegaly. Seventy-seven percent cases were below third centile for height and weight. Fetal haemoglobin was raised in all these cases ranging from 70 to 90 percent. Seventy six percent of the parents were heterozygous for this disease. Conclusion: In all these patients, there was poor compliance for blood transfusion and chelation therapy. The life expectancy in our patients is low as compared to western population.