Liver disease in patients with transfusion-dependent β-thalassemia: The emerging role of metabolism dysfunction-associated steatotic liver disease

Abstract

In this Editorial, we highlight the possible role that metabolism dysfunction-associated steatotic liver disease (MASLD) may play in the future, regarding liver disease in patients with transfusion-dependent β-thalassemia (TDBT). MASLD is characterized by excessive accumulation of fat in the liver (hepatic steatosis), in the presence of cardiometabolic factors. There is a strong correlation between the occurrence of MASLD and insulin resistance, while its increased prevalence parallels the global epidemic of diabetes mellitus (DM) and obesity. Patients with TDBT need regular transfusions for life to ensure their survival. Through these transfusions, a large amount of iron is accumulated, which causes saturation of transferrin and leads to the circulation of free iron molecules, which cause damage to vital organs (primarily the liver and myocardium). Over the past, the main mechanisms for the development of liver disease in these patients have been the toxic effect of iron on the liver and chronic hepatitis C, for which modern and effective treatments have been found, resulting in successful treatment. Additional advances in the treatment and monitoring of these patients have led to a reduction in deaths, and an increase in their life expectancy. This increased survival makes them vulnerable to the onset of diseases, which until recently were mainly related to the non-thalassemic general population, such as obesity and DM. There is insufficient data in the literature regarding the prevalence of MASLD in this population or on the risk factors for its occurrence. However, it was recently shown by a study of 45 heavily transfused patients with beta-thalassemia (Padeniya et al, BJH), that the presence of steatosis is a factor influencing the value of liver elastography and thus liver fibrosis. These findings suggest that future research in the field of liver disease in patients with TDBT should be focused on the occurrence, the risk factors, and the effect of MASLD on these patients.

Keywords: Metabolism dysfunction-associated steatotic liver disease; Transfusion-dependent thalassemia; Metabolic syndrome; Hepatic steatosis; Non-invasive markers; Liver fibrosis

Core Tip: Transfusion-dependent β-Thalassemia (TDBT) is one of the most frequent congenital diseases. Advances in the care of patients with TDBT over the past two decades have managed to significantly increase their life expectancy, making them more vulnerable to the onset of diseases related to the metabolic syndrome. Recent data from a relatively small study have highlighted the significance of the presence of hepatic steatosis in these patients. We herein discuss the available data concerning the presence of hepatic steatosis in this population, and the potential implications that these findings could have in the management of patients with TDBT, should these results be confirmed in larger cohorts.