Diagnosis and monitoring of iron overload in patients with haemoglobinopathies or rare anaemias
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چکیده
• Background Patients with haemoglobinopathies, such as β-thalassaemia and sickle cell disease (SCD), and other transfusion-dependent anaemias usually require regular blood transfusions which leads to iron accumulation in the heart and liver. Iron chelation therapy is used to prevent iron toxicity but requires close monitoring of body iron levels to ensure optimal dosage and avoid potential adverse effects of excess chelator treatment. The gold standard for evaluation of total body iron load is measurement of the liver iron concentration (LIC) via chemical analysis of liver needle biopsy specimens. However, liver biopsy is invasive, potentially associated with significant complications, costly and subject to sampling error.
منابع مشابه
Diagnosis and monitoring of iron overload in patients with haemoglobinopathies or rare anaemias
Background Patients with haemoglobinopathies, such as β-thalassaemia and sickle cell disease (SCD), and other transfusion-dependent anaemias usually require regular blood transfusions which leads to iron accumulation in the heart and liver. Iron chelation therapy is used to prevent iron toxicity but requires close monitoring of body iron levels to ensure optimal dosage and avoid potential adv...
متن کاملAustralian guidelines for the assessment of iron overload and iron chelation in transfusion-dependent thalassaemia major, sickle cell disease and other congenital anaemias.
Iron overload is the most important cause of mortality in patients with thalassaemia major. Iron chelation is therefore a critical issue in the management of these patients and others with transfusion-dependent haemoglobinopathies and congenital anaemias. In recent years, significant developments have been made in the assessment of iron overload, including the use of magnetic resonance imaging ...
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OBJECTIVES It is widely assumed that, at matched transfusional iron-loading rates, responses to chelation therapy are similar, irrespective of the underlying condition. However, data are limited for rare transfusion-dependent anaemias, and it remains to be elucidated if response differs, depending on whether the anaemia has a primary haemolytic or production mechanism. METHODS The efficacy an...
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Recently, some evidence has shown that the failure of iron homeostasis may occur in critically ill patients and can lead to iron overload. Elevated ferritin levels as a body iron burden index in critically ill patients may be associated with depressed level of consciousness and greater mortality. However, the necessity of using iron-chelating agents in clinical situation is still unknown for th...
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Background: Iron overload is caused early progression of atherosclerosis in beta thalassemia patients due to regular repeated blood transfusion. MRI T2* is a gold standard non-invasive method for detecting hepatic and cardiac iron overload. The aim of this study was the comparison of carotid intima media thickness (CIMT) in the patients and healthy control groups with Doppler ultrasound for ear...
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