A comparison of magnetic resonance imaging and cardiac biopsy in the evaluation of heart iron overload in patients with <i>β</i>‐thalassemia major

Mavrogeni, Sophie; Markussis, Vyron; Kaklamanis, Loukas; Tsiapras, Dimitrios; Paraskevaidis, Ioannis; Karavolias, George; Karagiorga, Markisia; Douskou, Marouso; Cokkinos, Dennis V.; Kremastinos, Dimitrios Th.

doi:10.1111/j.1600-0609.2005.00474.x

Cited by 88 publications

(70 citation statements)

References 34 publications

(43 reference statements)

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“…A number of studies have demonstrated that by measurement of T2* relaxation time MRI may also be eligible to measure iron overload in the heart, the most critical organ in this respect [28,34,35]. Deferasirox is assumed to be inferior to deferoxamine and deferiprone for the removal of excess cardiac iron [36][37][38], but there are no comparative data that exist in this regard.…”

Section: Discussionmentioning

confidence: 99%

Deferasirox in MDS patients with transfusion-caused iron overload—a phase-II study

et al. 2008

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International audienceBlood transfusions represent a main component of supportive care in myelodysplastic syndromes (MDS). To avoid organ damage caused by transfusion-dependent iron overload, an adequate iron chelation therapy is required. Recently, a new oral iron chelator deferasirox (ICL670, Exjade®) has become available. A study was conducted to demonstrate the efficacy and tolerability of deferasirox in transfusion-dependent iron-overloaded patients with MDS. The efficacy of deferasirox was monitored by changes in serum ferritin, bone marrow iron, and liver iron concentration (LIC), as determined by T2*-weighted magnetic resonance imaging. Twelve patients with MDS of different subtypes (median age 76 years, range 53–91) were enrolled. Deferasirox administered in a once-daily dose of 20–30 mg/kg for 12 months was effective in reducing median ferritin concentration from 1,515 µg/L (range 665–6,900) to 413 µg/L (range 105–3,052). Within the first 4 weeks of treatment before the continuous decline of ferritin levels, the values markedly rose in eight of 12 patients. The median LIC declined from 315 to 230 µmol/g ( = 0.02) at the end of study, accompanied by a reduction of bone marrow siderosis. The most common adverse events were mild and transient gastrointestinal disturbances, skin rash, nonprogressive transient increases in serum creatinine and urine β2-microglobulin, and a temporary reduction of the creatinine clearance. The renal parameters normalized after end of treatment. No hematologic toxicities were observed. Deferasirox proved to be effective in transfusion-dependent iron overload in MDS by mobilizing iron deposits in liver and at least stabilizing iron stores in bone marrow

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Section: Discussionmentioning

confidence: 99%

Deferasirox in MDS patients with transfusion-caused iron overload—a phase-II study

et al. 2008

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“…The development of the T2* MRI technique, which is today the reference standard for myocardial iron characterization, has opened up the opportunity to investigate myocardial iron overload in transfusion dependent adults in a non-invasive and reliable way. 11,19 In our series of patients, myocardial iron deposition developed after a heavy transfusion burden equal to or greater than 290 mL/kg of PRBC units corresponding to approximately 300 mg/kg of iron (101 units of PRBC in our series). This finding confirms historical post-mortem data by Buja.…”

Section: Discussionmentioning

confidence: 99%

Myocardial iron overload assessment by T2* magnetic resonance imaging in adult transfusion dependent patients with acquired anemias

Tucci¹,

Matta²,

Deplano³

et al. 2008

Haematologica

124

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Only limited data are available regarding myocardial iron overload in adult patients with transfusion dependent acquired anemias. To address this topic using MRI T2* we studied 27 consecutive chronic transfusion dependent patients with acquired anemias: (22 myelodysplastic syndrome, 5 primary myelofibrosis). Cardiac MRI T2* values obtained ranged from 5.6 to 58.7 (median value 39.8) milliseconds. Of the 24 analyzable patients, cardiac T2* correlated with transfusion burden (p=0.0002). No patient who had received less than 290 mL/kg of packed red blood cells (101 units=20 grams of iron) had a pathological cardiac T2* value (< 20 ms). All patients who had received at least 24 PRBC units showed MRI T2* detectable hepatic iron (liver T2* value ≤6.3 ms). Only patients with severe hepatic iron overload (T2* <1.4 ms) showed cardiac T2* value indicative of dangerous myocardial iron deposition. Serum ferritin was not significantly correlated with cardiac T2* (p=0.24). Gradient echo T2* magnetic resonance imaging provides a rapid and reproducible method for detecting myocardial iron overload which developed after a heavy transfusion burden equal to or greater than 290 mL/kg of packed red blood cell units.

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“…Owing to the lack of correlation between serum ferritin and myocardial iron content (Table 1), serum ferritin cannot be utilized in the assessment of myocardial iron content. In contrast, data from myocardial biopsy studies 13,15,17,18 reveal that cardiac T2 * relaxation times are in strong agreement with myocardial iron content quantified by myocardial biopsy. Mavrogeni et al 13 suggested that cardiac T2 * relaxation times <32 msec had 78% sensitivity and a specificity of 86% in predicting myocardial iron overload and excellent correlation with myocardial biopsy results.…”

Section: Myocardial T2 * and Cardiac Biopsymentioning

confidence: 40%

Quantification of Myocardial Iron Overload by Cardiovascular Magnetic Resonance Imaging T2* and Review of the Literature

Kondur

Vaitkevicius

et al. 2008

Clinical Cardiology

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Heart failure due to myocardial iron overload remains the leading cause of death in patients with transfusion‐dependent anemias. Iron overload‐induced cardiomyopathy is reversible if intensive chelation therapy is instituted on time. Thus, early detection of myocardial iron deposition is imperative to prevent overt heart failure. Conventional cardiac monitoring, including physical examination, electrocardiography, echocardiography or serum ferritin levels fail to predict manifest or subclinical myocardial involvement resulting from iron overload. Cardiovascular magnetic resonance imaging T2* (cMRI‐T2*, pronounced T2 star) times correlate well with myocardial iron levels. This timely review focuses on the utility of cMRI‐T2*, for the preclinical detection of myocardial iron overload and monitoring of myocardial iron content during chelation therapy. Copyright © 2008 Wiley Periodicals, Inc.

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A comparison of magnetic resonance imaging and cardiac biopsy in the evaluation of heart iron overload in patients with β‐thalassemia major

Abstract: Heart T2 relaxation time appears in agreement with cardiac biopsy, both in high and low iron deposition, and may become a useful non-invasive index in beta-thalassemia.

Cited by 88 publications

References 34 publications

Deferasirox in MDS patients with transfusion-caused iron overload—a phase-II study

Deferasirox in MDS patients with transfusion-caused iron overload—a phase-II study

Myocardial iron overload assessment by T2* magnetic resonance imaging in adult transfusion dependent patients with acquired anemias

Quantification of Myocardial Iron Overload by Cardiovascular Magnetic Resonance Imaging T2* and Review of the Literature

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