Survival and Disease Complication of Thalassemia Major: Experience of 14 Years at King Abdulaziz University

Abstract. Treatment of thalassemia major is complex, expensive and requires a multidisciplinary approach. Optimal clinical care is demanding and expensive, but achievable. To assess the prevalence of survival and disease complications among patients with thalassemia major treated at our center; a retrospective chart review was done of all patients followed and treated at King Abdulaziz University Hospital with a diagnosis of Thalassemia Major from 1990-2004. A total of 360 patients (203 males & 157 females) were transfusion dependant since early childhood and treated with parenteral Deferoxamine. Out of 360 patients, 293 (90.29%) patients were alive, 27 (7.2%) patients had died, 15 (4.2%) patients underwent BMT and 25 (6.9%) patient ’ follow-up were lost. Twelve (3.3%) patients died from heart disease. 7 (1.9%) patients died from infections, all patients were splenectomized. The serum ferritin levels for patients who died were significantly higher than for those patients who survived (7,500 vs. 3, 200; p < 0.001). Cardiac constitutes the first important cause of death followed by infection. Complications and deaths among thalassemics is iron related organ dysfunction and age related. The majority of complicated patients were on non-optimal chelation therapy and non-compliance