Low Serum Ferritin Levels are Misleading for Detecting Cardiac Iron Overload and Increase the Risk of Cardiomyopathy in Thalassemia Patients. The Importance of Cardiac Iron Overload Monitoring Using Magnetic Resonance Imaging T2 and T2*

Abstract

The incidence of cardiomyopathy was monitored in a 6-year follow-up study involving 56 transfused thalassemia patients treated with deferoxamine (DFO), deferiprone (L1) or their combination. During this period, five female patients on regular subcutaneous or intravenous DFO presented with cardiac complications. Three patients suffered congestive heart failure and the other two arrhythmias. Four of the five patients maintained serum ferritin levels of about 1 mg/L or below and the fifth about 1.5 mg/L for several years prior to the cardiomyopathy. Cardiac magnetic resonance imaging (MRI) T2* and T2 was performed in four patients after the cardiomyopathy, identifying the presence of moderate-to-heavy siderosis. The treatment of the five patients has since changed, involving mainly the use of L1. Low serum ferritin levels appear to be misleading for detecting cardiac iron overload and this may increase the risk of cardiomyopathy. The MRI T2 and T2* relaxation time measurements are a more accurate method of detecting cardiac iron overload. Chelation therapy using L1 or appropriate L1/DFO combinations can reduce cardiac iron overload and the mortality rate in thalassemia patients.

The relationship between myocardial t2* and left ventricular volumetric and functional parameters in thalassemia major patients

“…However, many unexplained deaths have occurred due to cardiac failure in TM patients despite low serum ferritin levels (29). The studies in the literature demonstrate that the relationship between the cardiac T2* value and the serum ferritin level is either non-significant or weak (10,30).…”

T2* cardiovascular magnetic resonance in the management of thalassemia patients in Oman

“…Kolnagou demonstrated that 5 out of 56 patients with good chelation compliance and low serum ferritin presented with unexpected cardiomyopathy. 10 Amongst our patients, one family of 4 siblings had exceptional compliance with deferoxamine monotherapy as shown by their average serum ferritin levels over the previous 13 years (Table 1B). One of them, MM, had presented in 2004 with cardiac failure with an ejection fraction of 36%.…”

Early detection of cardiac involvement in thalassemia: From bench to bedside perspective

“…Several studies in the last decade demonstrated that serum ferritin is not suitable for its use as a predictive indicator of myocardial iron deposition due to its lack of relationship with cardiac iron [17,18] . A recent study reported that many unexplained cardiac deaths in TM patients were found even though they had low serum ferritin levels [19] , emphasizing the unreliable use of serum ferritin as a predictor for iron overload cardiomyopathy in TM patients.…”