Hemochromatosis is defined as abnormal accumulation of iron in various organs of the body such as liver, pancreas, skin, joints, heart due to raised gut absorption of iron due to low hepcidin yield. Hemochromatosis is secondary to thalassemia major in this case. In the absence of other concurrent processes, cardiac hemochromatosis is identified as the presence of cardiac dysfunction owing to increased iron accumulation in the heart leading to heart failure.
Unique: Cardiac involvement in secondary hemochromatosis due to thalassemia major.
Take Away lesson: Regular monitoring of serum iron, ferritin, echocardiographic monitoring among thalassemia major patients is essential. Despite the fact that heart failure due to iron overload could be fatal, early diagnosis and intervention could prevent and treat the disease.