Magnetic resonance evaluation of liver and myocardial iron deposition in thalassemia intermedia and b-thalassemia major

Mavrogeni, Sophie; Gotsis, Efstathios D.; Ladis, Vassilios; Berdousis, Eleni; Verganelakis, Dimitrios; Toulas, Panagiotis; Cokkinos, Dennis V.

doi:10.1007/s10554-008-9332-2

Cited by 37 publications

(27 citation statements)

References 17 publications

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“…Although magnetic resonance with the T2* technique remains the gold standard for early diagnosis of cardiac hemochromatosis 3,6,7,36 , echocardiography can be used as a screening method. Due to its low cost and widespread availability, echocardiography is also a valuable instrument for monitoring patient evolution by permitting structural and functional cardiac parameter comparisons at different moments.…”

Section: Discussionmentioning

confidence: 99%

Ecocardiografia de pacientes talassêmicos sem insuficiência cardíaca em tratamento com transfusões sanguíneas e quelação

Rodrigues¹,

Guimarães-Filho²,

Braga³

et al. 2013

Arq. Bras. Cardiol.

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Section: Discussionmentioning

confidence: 99%

Ecocardiografia de pacientes talassêmicos sem insuficiência cardíaca em tratamento com transfusões sanguíneas e quelação

Rodrigues¹,

Guimarães-Filho²,

Braga³

et al. 2013

Arq. Bras. Cardiol.

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“…49 Interestingly, cardiac siderosis and subsequent cardiac disease do not seem to be a major concern in NTDT, even in patients with considerably elevated liver iron concentration. [50][51][52][53] In a recent cross-sectional study of 168 patients with β-thalassemia intermedia, higher liver iron concentration values on magnetic resonance imaging were associated with a significantly increased risk of developing thrombosis, pulmonary hypertension, hypothyroidism, hypogonadism, and osteoporosis. 46 Levels of 5 mg Fe/g or over were associated with a considerable morbidity risk increase.…”

Section: Dysregulated Iron Homeostasis and Clinical Iron Overloadmentioning

confidence: 99%

“…40,44,47,51 Therefore, spot measurements of serum ferritin level may underestimate iron overload and delay therapy in patients with NTDT if they are to be interpreted in the same way as β-thalassemia major patients. Although current evidence suggests that patients with NTDT are less likely to develop cardiac siderosis, [50][51][52][53] cardiac magnetic resonance T2* assessment may still be warranted in older patients with high iron burden. 37 Data on the use of other iron overload indices, such as transferrin saturation or non-transferrin bound iron, in NTDT patients are still limited; therefore, no recommendations regarding their use in clinical practice can be made at this time.…”

Section: Iron Chelation Therapymentioning

confidence: 99%

Non-transfusion-dependent thalassemias

Musallam

Rivella

Vichinsky³

et al. 2013

Haematologica

249

268

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“…Several case reports and case series also suggest an association between iron overload and hepatocellular carcinoma in this patient population (23)(24)(25). Interestingly, studies using cardiac magnetic resonance imaging (MRI) did not observe cardiac siderosis even in NTDT patients with substantially elevated LIC (26)(27)(28)(29). However, the number of patients recruited to these studies was small.…”

Section: Clinical Consequencesmentioning

confidence: 94%

“…Measurements can be done at one-or even two-year intervals (5), although closer monitoring may be needed to tailor therapy in patients eligible for iron chelation (LIC >5 mg Fe/g dw) (19). Although current evidence suggests that patients with NTDT are less likely to develop cardiac siderosis (26)(27)(28)(29), cardiac MRI T2* assessment may still be warranted in older patients with high LIC.…”

Section: Assessment Of Iron Overloadmentioning

confidence: 99%

Iron Overload in Non-Transfusion-Dependent Thalassemia

Musallam

2013

Thalassemia Reports

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Iron overload due to increased intestinal iron absorption remains a concern in patients with non-transfusion-dependent thalassemia (NTDT). A dynamic regulation between ineffective erythropiesis and iron metabolism in these disorders has been recently elucidated. Although the rate of iron loading in NTDT is slower than that observed in regularly transfused patients, the process is cumulative and patients may reach considerably high liver iron concentration levels. The clinical consequences of iron overload in patients with NTDT are various and include hepatic disease, endocrinopathy, bone disease, and vascular outcomes; while cardiac siderosis is less frequently observed. Although serum ferritin levels correlate with LIC in NTDT, they underestimate iron load when compared with transfusion-dependent patients with equivalent LIC. Therefore, direct measurement of LIC is recommended to identify patients at risk and guide iron chelation decisions. IntroductionTransfusion-dependence is an essential factor in characterizing the various phenotypes and their severity within the thalassemia syndromes. For instance, a diagnosis of b-thalassemia major entails lifelong transfusion requirement for normal growth and development, and more importantly, survival. The main concern with transfusiondependence is secondary iron overload, which if left untreated, leads to target-organ toxicity and death (1). However, considerable advances have been attained, especially in the last decade, in iron overload assessment and management strategies for transfusion-dependent patients; which translated into improved patient outcomes (2-3). Nontransfusion-dependent thalassemia (NTDT) is a term used to label patients who do not require such regular transfusions for survival and encompasses several entities, the most prevalent of which are a-thalassemia (mainly HbH disease), hemoglobin E/b-thalassemia, and bthalassemia intermedia (4). Despite their transfusion-independence, patients with NTDT are still at risk of iron overload (5). Only recently, data on the mechanisms, consequences, and assessment of iron overload in NTDT started to emerge; and these will be herein reviewed. PathophysiologyPatients with NTDT may still require occasional blood transfusions; for example, in the case of poor growth or development during childhood, during infection or pregnancy, prior to surgery, or for the management of specific complications where the benefit of transfusion therapy has been established (6). Blood transfusions in NTDT patients, however, contribute much less to the total iron burden than they do in transfusion-dependent patients, and the main source of iron remains primary loading, similar to patients with hereditary hemochromatosis. The combination of ineffective erythropoiesis, anemia, and hypoxia leads to a compensatory increase in serum levels of erythropoietin, as well as a decrease in serum levels of hepcidin, which control the concentration of ferroportin on the intestinal epithelium (7). Proposed regulators of hepcidin production incl...

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Magnetic resonance evaluation of liver and myocardial iron deposition in thalassemia intermedia and b-thalassemia major

Abstract: Although in TM iron plays a crucial role in the evolution of the disease, in TI the high output cardiac state seems to be the most prominent finding.

Cited by 37 publications

References 17 publications

Ecocardiografia de pacientes talassêmicos sem insuficiência cardíaca em tratamento com transfusões sanguíneas e quelação

Ecocardiografia de pacientes talassêmicos sem insuficiência cardíaca em tratamento com transfusões sanguíneas e quelação

Non-transfusion-dependent thalassemias

Iron Overload in Non-Transfusion-Dependent Thalassemia

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