Thalassemia Major/Cooley’s anaemia

Thalassemia Major/Cooley’s anaemia:
• genetic defect in synthesis of haemoglobin  ineffective erythropoiesis  microcytic, haemolytic anaemia
• more common n those of Mediterranean descent
• in addition to anaemia  skeletal deformities, hear failure, haemosiderosis
• X-ray – small bones of hand and feet appear tubular, coarse trabecular pattern and have cystic abnormalities.
• Management – frequent transfusions of packed RBC’s; iron chelation therapy; maybe splenectomy.