The Correlation of Cardiac and Hepatic Hemosiderosis as Measured by T2*MRI Technique with Ferritin Levels and Hemochromatosis Gene Mutations in Iranian Patients with Beta Thalassemia Major

Soltanpour, Mohammad Soleiman; Davari, Kambiz

doi:10.5001/omj.2018.09

Cited by 18 publications

(17 citation statements)

References 23 publications

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“…The latter should be made a priority given the large number of registered patients with significant iron overload, as serum ferritin may not accurately reflect the extent of iron overload, particularly cardiac overload. 29 …”

Section: Discussionmentioning

confidence: 99%

Complications and Challenges in the Management of Iraqi Patients with β-Thalassemia Major: A Single-center Experience

2020

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Objectives We sought to assess the complications and challenges facing the management of β-thalassemia major (β-TM) in Iraq. Methods A total of 150 consecutive patients with β-TM who were registered at a main thalassemia center in Northern Iraq were enrolled in the study. The patients had their records reviewed, were clinically evaluated, and investigated for various complications. Results Our patient cohort had a median age of 13 years (range: 1–35 years) and a male to female ratio of 1:1.2. Their median serum ferritin was 2762 µg/L, all were on regular transfusions, 94.7% were on chelation therapy, and 38.0% were splenectomized. Pre-transfusion hemoglobin levels were 3 9.0 g/dL in 38.7% of the patients. Short stature was encountered in 33.9% of those aged ≤ 20 years, and skeletal changes were noted in 50.7%. Iron overload associated complications, including hypogonadism, hypothyroidism, hypoparathyroidism, diabetes mellitus, and heart failure, were encountered in 52.8%, 7.3%, 3.3%, 3.3%, and 2.7%, respectively. Hepatitis C virus (HCV) antibodies were detectable in 35.3%, while HIV antibodies and hepatitis B surface antigen were not detectable in any. Patients with diabetes mellitus, heart failure, HCV antibodies, and hypoparathyroidism were significantly older than those without these complications. Hypogonadism was the only complication associated with significantly higher serum ferritin levels. Hypogonadism, heart failure, HCV antibodies, and diabetes were significantly more frequent among the splenectomized patients. Conclusions The management of β-TM in this cohort of Iraqi patients is still suboptimal, and the need to ensure timely transfusions and optimize chelation, as well as a more robust iron overload assessment, should be underscored.

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Section: Discussionmentioning

confidence: 99%

Complications and Challenges in the Management of Iraqi Patients with β-Thalassemia Major: A Single-center Experience

2020

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“…For T2* and MIC analysis, a homogeneous full-thickness region of interest (ROI) was chosen in the septum30 The signal intensity of this region was measured for each of the images and the data were plotted against the TE to form an exponential decay curve. Cut-off points in this MRI instrument are as follows: Cardiac: normal >20ms, mild: 14–20 ms, moderate: 10–14 ms, severe <10 ms; Liver: normal >6.3 ms, mild: 2.8–6.3 ms, moderate: 1.4–2.7 ms, severe <1.4 ms 12…”

Section: Methodsmentioning

confidence: 99%

“…Therefore, myocardial iron measurement is an important step in determining the risk of cardiac complications 11. and in tailoring the appropriate iron-chelation treatment for transfused thalassemia major patients 4,12. Cardiac T2* magnetic resonance imaging (MRI), using an intense magnetic field, can successfully assess iron deposition in cardiac muscles.…”

Section: Introductionmentioning

confidence: 99%

<p>A randomized controlled trial evaluating the effects of amlodipine on myocardial iron deposition in pediatric patients with thalassemia major</p>

Khaled¹,

Salem²,

Ezzat³

et al. 2019

DDDT

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Background Mortality rates increase due to iron deposition in the cardiac muscles of thalassemia major (TM) patients. Iron overload cardiomyopathy could be treated with a combination therapy of an iron chelator and an L-type calcium channel blocker. We designed a randomized controlled study to assess the potential of amlodipine, alongside chelation, in reducing myocardial iron concentration in TM patients compared with a placebo. Objectives This study aims to estimate the change in myocardial iron concentration (MIC) determined by magnetic resonance imaging after 6 months of treatment with amlodipine, as well as measuring the changes in the secondary outcomes (liver iron concentration (LIC), serum ferritin level (SF), and left ventricle ejection fraction (LVEF)) of study participants. Methods A single, randomized, placebo-controlled trial was performed in 40 β-Thalassemia major patients aged between 6 and 20 years old, who received either oral amlodipine 2.5–5 mg/day or a placebo, in addition to a Deferasirox chelation regimen in a 1:1 allocation ratio. Results After 6 months, a significant reduction was noted in the MIC of patients receiving amlodipine (n=20), compared with the patients receiving the placebo (n=20). At baseline, the mean was 0.76±0.11 mg/g dry weight, while at 6 months, the mean was 0.51±0.07 mg/g dry weight ( p <0.001). Also, there was a significant change in the myocardial T2* after 6 months; the amlodipine increased the myocardial T2* from 40.63±5.45 ms at baseline to 43.25±5.35 ms ( p <0.001). However, amlodipine did not significantly affect the secondary outcomes by the end of the study. Conclusion The addition of amlodipine to the standard chelation therapy in transfusion-dependent thalassemia major patients improves myocardial iron overload without increasing the adverse effects.

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“…Currently, the best noninvasive method to evaluate target organ hemosideriosis is T2* magnetic resonance imaging 12. However, several factors hinder the use of MRI; it is difficult to use with children, requires an expert radiologist to interpret its results, is not available in all locations, and is expensive 13…”

Section: Introductionmentioning

confidence: 99%

<p>Effective method of evaluating myocardial iron concentration in pediatric patients with thalassemia major</p>

Khaled¹,

Ezzat²,

Salem³

et al. 2019

JBM

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Background The use of T2* magnetic resonance imaging (MRI) has been promoted by recent studies as a noninvasive method for the detection of iron overload in thalassemia major patients. This study aims to estimate the iron load in the heart and liver of thalassemia major patients using T2* MRI and to determine its correlation with the left ventricle ejection fraction and serum ferritin level. Methods Forty β-Thalassemia major patients were included in the study. We evaluated the serum ferritin level, echocardiography, cardiac T2*, myocardial iron concentration (MIC), liver iron concentration (LIC) and hepatic T2* in all patients. CMR T2* findings were categorized as normal cardiac T2* (T2* >20 ms) or abnormal cardiac T2* (T2* <20 ms). Results The study found that 85% of patients had a normal cardiac T2* value. The median serum ferritin level was 2189. A significant inverse correlation was found between the serum ferritin level and the cardiac T2* (r=−0.381, =0.015); however, the correlations between serum ferritin and the hepatic T2* and liver iron concentration were statistically non-significant ( P =0.539 and P =0.637, respectively). Additionally, the LVEF correlation was statistically non-significant with SF, hepatic T2* and cardiac T2*. Conclusion Regardless of the serum ferritin level or left ventricle function, a cardiac T2* MRI should be done for all patients with β-Thalassemia major in order to estimate the myocardial iron concentration.

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The Correlation of Cardiac and Hepatic Hemosiderosis as Measured by T2*MRI Technique with Ferritin Levels and Hemochromatosis Gene Mutations in Iranian Patients with Beta Thalassemia Major

Cited by 18 publications

References 23 publications

Complications and Challenges in the Management of Iraqi Patients with β-Thalassemia Major: A Single-center Experience

Complications and Challenges in the Management of Iraqi Patients with β-Thalassemia Major: A Single-center Experience

<p>A randomized controlled trial evaluating the effects of amlodipine on myocardial iron deposition in pediatric patients with thalassemia major</p>

<p>Effective method of evaluating myocardial iron concentration in pediatric patients with thalassemia major</p>

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