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.
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