Abstract
Iron can be deposited in all internal organs, leading to different types of functional abnormalities. However, myocardial iron overload that contributes to heart failure remains one of the main causes of death in thalassemia major. Using magnetic resonance imaging, tissue iron is detected indirectly by the effects on relaxation times of ferritin and hemosiderin iron interacting with hydrogen nuclei. The presence of iron in the human body results in marked alterations of tissue relaxation times. Currently, cardiovascular magnetic resonance using T2* is routinely used in many countries to identify patients with myocardial iron loading and guide chelation therapy, specifically tailored to the heart. Myocardial T2* is the only clinically validated non-invasive measure of myocardial iron loading and is superior to surrogates such as serum ferritin, liver iron, ventricular ejection fraction and tissue Doppler parameters. Finally, the substantial amelioration of patients’ survival, allows the detection of other organs’ abnormalities due to iron overload, apart from the heart, missed in the past. Recent studies revealed that iron deposition has a different pattern in various parenchymal organs, which is independent from serum ferritin and follows an individual way after chelation treatment application. This new upcoming reality orders a closer monitoring of all organs of the body in order to detect preclinical lesions and early apply adequate treatment.
Highlights
Over 300,000 children are born each year with a hemoglobinopathy, of whom, more than 25,000 have thalassemia major (TM) and need regular transfusions to survive beyond early childhood
Myocardial iron overload ly that contributes to heart failure remains one of the main causes of death in thalassemia n major
Tiso sue iron is detected indirectly by the effects on relaxation times of ferritin and hemosiderin e iron interacting with hydrogen nuclei
Summary
Iron can be deposited in all internal organs, causing different types of functional abnormalities. The measurement of plasma ferritin provides an indirect index for the total body iron stores, but its usefulness is limited by many common clinical conditions, such as inflammation, fever or liver disease.[5] It fails to reflect myocardial iron overload.[6,7,8,9] Liver biopsy suffers from the same problems, mostly owing to the fact that iron deposition occurs in the liver prior to and at a greater scale than that of the correlated strongly with heart T2*. The lack of availability, cost, technical demands, unsatisfactory correlation with biopsy, lack of heart-relevant data and suboptimal reproducibility have restricted the clinical use of the method.[13] it is clear that there is an emerging need for a non-invasive, reproducible index, capable of accurate detection of iron in an individual organ and in an individual patient
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