5610178 SURVIVAL AND COMPLICATIONS IN PATIENTS WITH HEMOGLOBINOPATHIES: THE EXPERIENCE OF A SINGLE DEPARTMENT OF THALASSEMIA AND SICKLE DISEASE

Abstract

Background: New medicines and doctors’ expertise have increased hemoglobinopathies patients’ survival from 35 to 60 years in the last few decades. This report presents the underlying causes of death in a thalassemia and sickle cell disease unit during the past decade and examines potential risk factors. All causes of death were reported and correlated with comorbidities that may have contributed to this outcome. Methods: The records of 32 patients (17 women/15 men) who died in the Thalassemia and Sickle Cell Disease Unit in the last 10 years were reviewed: 11 had thalassemia, 10 had thalassemia/sickle cell disease, 9 had NTD, and 2 had sickle cell disease. Patients died at a median age of 42 years (range 29-71). Results: Heart failure (8, 25%), HCC (72%), and sepsis (6, 19%) were the leading reasons of mortality.The immediately subsequent causes of death that were recorded were as follows: sudden death three cases (9.5%), liver failure three cases (9.5%), brain aneurysm one case (1%), motor neuron disease one case (1%), and an car accident one time respectively. Using the Pearson Correlation Coefficient, a positive correlation (r:0.8) was found between deaths from heart failure, HCC, and liver failure in patients with a high iron load, as well as a moderate correlation (r:0.3) of HCV infection in patients who died of HCC and liver failure, with no correlation in the remaining deaths. Similarly, no death was associated with splenectomy or the number of transfusions. Conclusions: Despite improvements in better treatment options, heart failure and HCC caused by iron overload continue to be major causes of morbidity and mortality in people with hemoglobinopathies.