Cardiomyopathy in Thalassemia: Quick Review From Cellular Aspects to Diagnosis and Current Treatments Publisher Pubmed



Ghanavat M¹ ; Haybar H² ; Pezeshki SMS³ ; Shahjahani M³ ; Jodat H³ ; Elyasi M³ ; Saki N^{1, 3} Show Affiliations
Source: Lab Medicine Published:2020

Abstract

Background: Cardiomyopathic manifestations induced by continuous blood transfusion are the leading cause of death among patients with thalassemia major (TM). Despite introduction of chelation therapy, heart failure after cardiomyopathic manifestations is still a major threat to patients. Methods: We performed a search of relevant English-language literature, retrieving publications from the PubMed database and the Google Scholar search engine (2005-2018). We used thalassemia major, cardiomyopathy, iron overload, cardiac magnetic resonance T2 chelation therapy, and iron burden as keywords. Results: The results of the studies we found suggest that cardiac hepcidin is a major regulator of iron homeostasis in cardiac tissue. Unlike previous assumptions, the heart appears to have a limited regeneration capability, originating from a small population of hypoxic cardiomyocytes. Conclusions: Oxygen levels determine cardiomyocyte gene-expression patterns. Upregulation of cardiac hepcidin in hypoxia preserves cardiomyocytes from forming out of reactive oxygen species catalyzed by free cellular iron in cardiomyocytes. Using the limited regeneration capacity of cardiac cells and gaining further understanding of the cellular aspects of cardiomyopathic manifestations may help health care professionals to develop new therapeutic strategies. © 2019 American Society for Clinical Pathology. All rights reserved.