Burden of Cardiac Siderosis in a Thalassemia-Major Endemic Population: A Preliminary Report From Pakistan

Abstract

There is a high prevalence of severe myocardial iron load in Pakistani TM patients. Serum ferritin did not correlate with T2* values. Lower T2* was the only clinical factor associated with the NYHA functional class.

Clinical utility of endocrine markers predicting myocardial siderosis in transfusion dependent thalassemia major

“…14,24 The high prevalence of endocrinopathies in our population is likely due to the higher prevalence of severe iron overload, as reflected by T2* CMR. This is demonstrated by the fact that 60% of our patients had a T2* CMR value of <10 msec, findings that are in line with what we reported in our preliminary study 12 ; this degree of iron overload is much higher compared to international and regional prevalence, which ranges from 25% to 44%. [25][26][27] This iron overload is not only a reflection of the inadequately managed chelation therapy in our setting 26 but also of the delayed age of presentation of our patients (17 years [IQR: 7 years]); increasing age has been shown to be a risk factor for developing endocrinopathies.…”

“…Due to its poor correlation with T2* CMR and its variation during inflammation, there is a need for an alternative marker to guide chelation. This especially holds true in populations such as ours where there is a high prevalence of blood transfusion transmitted infections . Hence, serum ferritin has little to no value in the assessment of myocardial siderosis.…”

“…This especially holds true in populations such as ours where there is a high prevalence of blood transfusion transmitted infections. 12,[33][34][35] Hence, serum ferritin has little to no value in the assessment of myocardial siderosis.…”

“…Cardiac iron load was considered normal if the T2* CMR value was >20 msec, mild to moderate if T2* CMR = 10–20 msec, and severe myocardial siderosis if the T2* CMR value was <10 msec . Iron overload based on serum ferritin levels > 2500 μg/l, an average of minimum one and maximum five recent values, whichever was available, was considered severe, and measured via standard enzyme immunoassay at the different centers (Ferritin Calibrator; Hoora Pharma, and Immulite 2000 Processor, Siemens Healthcare Global, Erlangen, Germany).…”

“…The preliminary T2* CMR based study from Pakistan reported that 47% of our TDTM population had severe myocardial siderosis. 12 Significant endocrinopathies, especially hypogonadism and hypoparathyroidism, 13,14 have also been reported. The majority of these patients are suboptimally chelated.…”