Iron Status in Patients with Diamond Blackfan Anemia

Abstract

The comparison of the severity of iron overload effect in beta thalassemia major (BTM) and transfusion dependent Diamond-Blackfan anemia (DBA) is a complex issue. In the current study we aimed to assess the iron overload status using serum ferritin and magnetic resonance T2* imaging in transfusion dependent DBA patients as compared to BTM patients with. This study was conducted on 22 patients with DBA and 27 age and sex matched patients with beta thalassemia major serving as control. All subjects were recruited from the Pediatric Hematology Clinic, of Ain Shams University Children’s Hospital. All subjects were subjected to full detailed medical history taking and revision of the hospital records laying stress on age at diagnosis, age at study entry, sex, consanguinity, family history, medications given including steroids or others and transfusion history including age at first transfusion and calculation of the transfusion index. Thorough clinical examination was done including assessment of anthropometric measurements including weight in kilograms (Kg), height in centimeters (cm), and body mass index with calculation of standard deviation score for age and sex (SDS) and Complete examination including cardiac, chest, abdominal, and neurological examination for the presence of congenital anomalies and complications of disease or therapy. Diagnostic workup: Initial CBC, WBC, reticulocyte count, Serum ferritin and Cardiac and hepatic iron loading was quantified by means of the MRI T2* technique.