MRI guided iron assessment and oral chelator use improve iron status in thalassemia major patients

Abstract

Oral iron chelators and magnetic resonance imaging (MRI) assessment of heart and liver iron burden have become widely available since the mid 2000s, allowing for improved patient compliance with chelation and noninvasive monitoring of iron levels for titration of therapy. We evaluated the impact of these changes in our center for patients with thalassemia major and transfusional iron overload. This single center, retrospective observational study covered the period from 2005 through 2012. Liver iron content (LIC) was estimated both by a T2* method and by R2 (Ferriscan®) technique. Cardiac iron was assessed as cT2*. Forty-two patients (55% male) with transfused thalassemia and at least two MRIs were included (median age at first MRI, 17.5 y). Over a mean follow-up period of 5.2 ± 1.9 y, 190 MRIs were performed (median 4.5 per patient). Comparing baseline to last MRI, 63% of patients remained within target ranges for cT2* and LIC, and 13% improved from high values to the target range. Both the median LIC and cT2* (cR2* = 1000/cT2*) status improved over time: LIC 7.3 to 4.5 mg/g dry weight, P = 0.0004; cR2* 33.4 to 28.3 Hz, P = 0.01. Individual responses varied widely. Two patients died of heart failure during the study period. Annual MRI iron assessments and availability of oral chelators both facilitate changes in chelation dose and strategies to optimize care.

Effect of iron overload on impaired fertility in male patients with transfusion-dependent beta-thalassemia

“…deposition in myocardium, liver, and brain tissue in patients with iron overload, and has been validated by histologic evaluation (32)(33)(34). The MRI T2 and R2 techniques have also been applied to determine the severity of the iron burden causing liver and cardiac injury, and to evaluate the efficacy of iron chelator treatment in patients with thalassemia major (23,35). A small series of seven male patients with transfusion-dependent thalassemia that compared the MRI R2 value of the anterior pituitary gland to sperm quality reported a relatively higher MRI R2 value of the anterior pituitary gland in male patients with concurrent high ferritin and low FSH/LH levels in three patients with azoospermia (14).…”

Guidelines for the Standard Monitoring of Patients With Thalassemia

“…If transfusion volumes and chelation history are known and the ferritin is in an acceptable range, the initial LIC measurement may be deferred until age 5 years. The LIC is usually monitored annually, and this practice led to changes in chelation after 40% of MRI studies [20]. More frequent assessments (every 6 months) may be considered when the iron burden is high and intensive chelation is utilized.…”

Effects of deferasirox-deferoxamine on myocardial and liver iron in patients with severe transfusional iron overload

“…Advances in the management of cardiac complications have led to a longer life expectancy in patients with transfusion-dependent anemias, 29,30 and other complications, including those related to liver iron overload, are increasingly being seen as a cause of death. 31,32 Therefore, the rapid and clinically significant decrease in liver iron burden from severe levels at baseline in HYPERION is important.…”