Modern management of iron overload in thalassemia major patients guided by MRI techniques: real-world data from a long-term cohort study

Bayraktaroğlu, Selen; Karadaş, Nihal; Onen, Sebnem; Karapınar, Deniz Yılmaz; Aydınok, Yeşim

doi:10.1007/s00277-021-04748-w

Cited by 7 publications

(8 citation statements)

References 33 publications

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“…Our study is consistent with previous reports of strong correlation between SF and liver IO. 3 , 18 , 23 – 26 We found that SF cut-off levels of 700 ng/mL and <1250 ng/mL effectively excluded mild and moderate hepatic IO, respectively with an LR− of 0.1. SF levels of 1650 ng/mL and 2150 ng/mL, on the other hand, strongly predicted mild and moderate hepatic IO with an LR+ of 13.9 and 10, respectively.…”

Section: Discussionmentioning

confidence: 78%

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Optimized serum ferritin prediction of iron overload in transfusion-dependent thalassemia: likelihood ratio and age-adjustment approach

Kurban

Almarri²,

Alshamsi³

et al. 2023

Ann Saudi Med

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BACKGROUND: Early detection of iron overload in transfusion-dependent thalassemia (TDT) patients is critical to prevent complications and improve survival. OBJECTIVES: Evaluate the utility of serum ferritin (SF) in the prediction of hepatic and myocardial iron overload (HIO and MIO) compared to T2*-MRI. DESIGN: Retrospective SETTINGS: Governmental hospitals. PATIENTS AND METHODS: Patients with TDT who had T2*-MRI examinations between January 2016 to October 2019 were included. The predictive value of SF for detection of HIO and MIO was assessed by measuring area under the curve (AUC). A sample size of 123 cases was calculated to detect a correlation of 0.25 with 90% power and a two-sided type I error of 0.05. MAIN OUTCOME MEASURES: The correlation between SF and estimated hepatic iron concentration. SAMPLE SIZE: 137 TDT patients who required regular blood transfusions. RESULTS: The predictive value of SF was excellent for detection of HIO (AUC=0.83-0.87) but fair for detection of MIO (AUC=0.67). The two independent predictors of MIO were age and SF. The log of (age × SF) enhanced the SF predictive value for MIO (AUC=0.78). SF values of 700 and 1250 mg/L effectively excluded mild and moderate HIO with a sensitivity of 97.8% and 94.2%, respectively (LR−=0.1). While SF values of 1640 and 2150 mg/L accurately diagnosed mild and moderate HIO with a specificity of 95.55% and 96.4%, respectively (LR+>10). A log of (age × SF) cut-off value of 4.15 effectively excluded MIO (LR−=0.1), while a value of 4.65 moderately confirmed MIO (LR+=3.2). CONCLUSIONS: SF is an excellent predictor of hepatic IO in TDT. Age adjustment enhanced its myocardial IO predictive accuracy. Likelihood ratio-based SF cut-off values may help clinicians in risk stratification and treatment decision-making. LIMITATIONS: The laboratory data were gathered retrospectively and although the risk of selection bias for T2*-MRI examination is thought to be low, it cannot be ignored. CONFLICT OF INTEREST: None.

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Section: Discussionmentioning

confidence: 78%

“…Other studies found different correlation strengths, ranging from no correlation to stronger correlation quoting different SF cut-off values for diagnosis of myocardial IO. 15 , 16 , 26 …”

Section: Discussionmentioning

confidence: 99%

Optimized serum ferritin prediction of iron overload in transfusion-dependent thalassemia: likelihood ratio and age-adjustment approach

Kurban

Almarri²,

Alshamsi³

et al. 2023

Ann Saudi Med

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“…However, arti cial delineation of ROI is susceptible to a variety of subjective and objective factors, the measurement error is inevitable. (2) The data used in this study are single-center data. Due to the characteristics of endemic sources, the proportion of patients with high iron concentration is relatively large, and the proportion of patients with normal or low iron concentration is relatively small.…”

Section: Discussionmentioning

confidence: 99%

“…The liver is one of the main iron storage organs, and LIC re ects the total iron load and is an important clinical indicator for clinical monitoring, evaluation and treatment of iron overload [1]. Although the actual liver iron concentration provided by liver biopsy serves as the "gold standard" for clinical indicators, most scholars and medical centers are more inclined to use non-invasive MRI technology to monitor LIC due to the fact that biopsy only provides a small sample size of LIC and has some disadvantages such as invasiveness and poor repeatability [2]. The LIC F reported by FerriScan (Resonance Health Limited, Burswood, WA, Australia) based on MRI T2/R2 (1000/T2) images has been certi ed by the US Food and Drug Administration (FDA) and has high reliability [3,4].…”

Section: Introductionmentioning

confidence: 99%

Study on the Equation Between Liver MRI T2* Values and Liver Iron Concentration in Thalassemia Patients

Liang

et al. 2022

Preprint

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Background: The liver iron concentration (LICF) provided by FerriScan is already certified. But there are some restrictive factors. Therefore, we explored the relationship curves of LICF and MRI liver T2 *, and constructed the equations for both. Methods: Liver MRI T2* values of 273 thalassemia patients were measured by CMRtools/Thalassemia Tools (CMRtools) and divided into test and verification groups. The T2* values of the test group and LICF were used to build the equation, through which the T2* values of the validation group were converted to the liver iron concentration（LICe）. The relationship between LICe and LICF was explored. According to the clinical liver iron concentration grading, LICF and LICe were grouped to explore the relationship between the them in the validation group. Results: The equation built by the test group was LICF=37.393T2*^(-1.22)（R2=0.971，P＜0.05）. There was no statistical difference between LICe and LICF in the validation group（P＞0.05）; There was significant consistency（W=0.991, P＜0.05）and significant correlation（rs=0.983，P＜0.05） between them .There was no statistical difference in the clinical grading between LICe and LICF in the validation group （P＞0.05）. There was significant consistency between the clinical grading results（K=0.943，P＜0.05. Conclusion: Through the equation LICF=37.393T2*^(-1.22), after measuring the liver T2* value, the liver iron concentration (LIC) equivalent to LICF can be accurately calculated.

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“…It was impressively demonstrated for patients with thalassemia major in different British birth cohorts that with the introduction of Magnetic Resonance Imaging (MRI) methods for the investigation of cardiac IOL around the year 2000 and the new possibility of tailoring therapy including the combination of chelators, there was a dramatic decrease in deaths from cardiac IOL-related complications [1]. More recently, another cohort study in patients with thalassemia major proved the importance of regular monitoring including myocardial and liver iron content (LIC) assessment to guide treatment of IOL [2].…”

Section: Introductionmentioning

confidence: 99%

Current German Guidelines on Diagnosis and Treatment of Secondary Hemochromatosis in Patients with Congenital Anemias

Cario

Grosse²,

Jarisch

et al. 2022

Klin Padiatr

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Adequate diagnosis and treatment of secondary hemochromatosis in patients with congenital anemias is important for reducing long-term mortality and morbidity as for improving patients' quality of life. Due to the strong migration movements during recent years, the number of patients in Germany suffering from hemoglobinopathies as some of the most relevant disorders in the context of iron overload (IOL) has increased enormously. Many of these patients had received inadequate medical care in their countries of origin prior to migration, including diagnosis and treatment of IOL. In parallel, various medical developments and achievements took place, including the expansion of stem cell transplant as curative therapeutic option and the introduction of gene therapy for patients with hemoglobinopathies. Diagnostic tools to assess both liver and heart IOL became available at more sites in Germany. Overall experience with iron elimination therapy either as monotherapy or as combination of different chelators increased. All these aspects were considered during revision of the consensus-based guideline on the diagnosis and treatment of secondary IOL in patients with congenital anemias (AWMF Reg. No. 025/029). Here, we briefly summarize the procedure for the revision of the guideline, provide a brief overview of innovations as compared to the previous version dated from 2015, and finally present the consensus recommendations adopted in the current version.

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Modern management of iron overload in thalassemia major patients guided by MRI techniques: real-world data from a long-term cohort study

Cited by 7 publications

References 33 publications

Optimized serum ferritin prediction of iron overload in transfusion-dependent thalassemia: likelihood ratio and age-adjustment approach

Optimized serum ferritin prediction of iron overload in transfusion-dependent thalassemia: likelihood ratio and age-adjustment approach

Study on the Equation Between Liver MRI T2* Values and Liver Iron Concentration in Thalassemia Patients

Current German Guidelines on Diagnosis and Treatment of Secondary Hemochromatosis in Patients with Congenital Anemias

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