Cardiac, Tissue Iron Differences Detected Between Thalassemia Intermedia, Major

In a study comparing thalassemia intermedia and thalassemia major, patients with thalassemia intermedia were found to have lower cardiac and pancreatic iron burden, along with more pronounced left ventricular hypertrophy. This is according to findings from a multicenter, retrospective, population-based study published in the Journal of Clinical Medicine.

“These differences could not be explained by the different duration of the transfusional regimen,” the study investigators noted.

The study involved a comparison of magnetic resonance imaging (MRI) findings in tissue between 135 adult, regularly transfused patients with thalassemia intermedia and an age- and sex-matched cohort of 135 patients with thalassemia major. There were 77 female patients in both cohorts.

The MRI protocol used to compare the two patient cohorts included the R2* technique for quantifying cardiac, hepatic, and pancreatic iron levels. It also evaluated parameters of biventricular function and monitored for replacement myocardial fibrosis.

A comparison of the two cohorts found no difference in hepatic iron levels (P=.853) and no significant differences between them regarding patient age, sex, frequency of splenectomy and chelation, or serum ferritin levels (P>.05).

Compared with patients who had thalassemia major, patients with thalassemia intermedia began their regular transfusions noticeably later (P<.0001). They also had significantly lower pre-transfusion hemoglobin levels (P=.005), cardiac R2* values (P<.0001), and pancreatic R2* values (P<.0001).

Patients with thalassemia intermedia had noticeably higher left ventricular mass index (P=.049) than those with thalassemia major. They also had significantly higher left and right ventricular end-diastolic volume indexes, though applying a correction for the duration of regular transfusions then removed disease-specific differences (P>.05).

No difference was found between the two cohorts (P>.05) in either biventricular ejection fractions or the degree of replacement myocardial fibrosis.

Regarding the comparison of the two cohorts, “[i]n the receiver operating characteristic analysis, pancreatic iron was the strongest discriminator between the two diseases,” the authors said.

Reference

Meloni A, Pistoia L, Ricchi P, et al. Magnetic resonance evaluation of tissue iron deposition and cardiac function in adult regularly transfused thalassemia intermedia compared with thalassemia major patients. J Clin Med. 2024;13(16):4791. doi:10.3390/jcm13164791