Transfusional hemosiderosis and combined chelation therapy in sickle thalassemia

Tsironi, Maria; Polonifi, Katerina; Deftereos, Spyros; Farmakis, Dimitrios; Andriopoulos, Panagiotis; Moyssakis, Ioannis; Aessopos, Athanasios

doi:10.1111/j.1600-0609.2005.00528.x

Cited by 14 publications

(18 citation statements)

References 14 publications

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“…Continuous desferrioxamine infusions alone, have been shown to improve cardiac function and salvage patients81 and is the treatment of choice if combination therapy is contraindicated. We have reported two cases with severe CCF who reversed with intensive combination therapy82,83 and we have at least 8 more patients with similar outcome. Two other studies show similar responses 84,85.…”

Section: Chelation Treatment For Prevention and Treatment Of Iron Indmentioning

confidence: 71%

Cardiac Function and Iron Chelation in Thalassemia Major and Intermedia: A Review of the Underlying Pathophysiology and Approach to Chelation Management

Aessopos

Berdoukas

2009

Medit J Hemat Infect Dis

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Heart disease is the leading cause of mortality and one of the main causes of morbidity in beta-thalassemia. Patients with homozygous thalassemia may have either a severe phenotype which is usually transfusion dependent or a milder form that is thalassemia intermedia. The two main factors that determine cardiac disease in homozygous β thalassemia are the high output state that results from chronic tissue hypoxia, hypoxia-induced compensatory reactions and iron overload. The high output state playing a major role in thalassaemia intermedia and the iron load being more significant in the major form. Arrhythmias, vascular involvement that leads to an increased pulmonary vascular resistance and an increased systemic vascular stiffness and valvular abnormalities also contribute to the cardiac dysfunction in varying degrees according to the severity of the phenotype. Endocrine abnormalities, infections, renal function and medications can also play a role in the overall cardiac function. For thalassaemia major, regular and adequate blood transfusions and iron chelation therapy are the mainstays of management. The approach to thalassaemia intermedia, today, is aimed at monitoring for complications and initiating, timely, regular transfusions and/or iron chelation therapy. Once the patients are on transfusions, then they should be managed in the same way as the thalassaemia major patients. If cardiac manifestations of dysfunction are present in either form of thalassaemia, high pre transfusion Hb levels need to be maintained in order to reduce cardiac output and appropriate intensive chelation therapy needs to be instituted. In general recommendations on chelation, today, are usually made according to the Cardiac Magnetic Resonance findings, if available. With the advances in the latter technology and the ability to tailor chelation therapy according to the MRI findings as well as the availability of three iron chelators, together with increasing the transfusions as need, it is hoped that the incidence of cardiac dysfunction in these syndromes will be markedly reduced. This of course depends very much on the attention to detail with the monitoring and the cooperation of the patient with both the recommended investigations and the prescribed chelation.

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Section: Chelation Treatment For Prevention and Treatment Of Iron Indmentioning

confidence: 71%

Cardiac Function and Iron Chelation in Thalassemia Major and Intermedia: A Review of the Underlying Pathophysiology and Approach to Chelation Management

Aessopos

Berdoukas

2009

Medit J Hemat Infect Dis

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“…Given also the high-output state, it is obvious that the left ventricle is in a continuous state of both volume and pressure overload. Myocardial iron load seems of less importance in sickling syndromes compared to thalassemia major [8,29,30]. However, in 50% of S-Thal patients previously studied by CMR, mild iron overload was encountered, despite the absence of regular transfusion therapy [18].…”

Section: Discussionmentioning

confidence: 99%

Cardiac involvement in sickle β-thalassemia

et al. 2008

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Cardiovascular involvement is a leading cause of mortality and morbidity in patients with inherited hemoglobinopathies, but it has not been adequately assessed in sickle β-thalassemia. We evaluated 115 sickle β-thalassemia patients, aged 34±14 years, along with 50 healthy controls, by resting echocardiography. Patients with systolic left ventricular (LV) dysfunction or severe pulmonary hypertension (PHT) also underwent left and right cardiac catheterization and cardiac magnetic resonance imaging (CMR). Left and right chamber dimensions, LV mass, and cardiac index were significantly higher in patients compared to controls (p<0.001 in most cases). Three patients (2.9%) had reduced LV ejection fraction (<55%); mean LV ejection fraction was significantly lower in patients (p< 0.001). Left and right ventricular systolic tissue Doppler indices and LV diastolic tissue Doppler indices were also impaired in patients. All three patients with systolic LV dysfunction had normal coronary arteries and mild myocardial iron load (CMR T2* values, 18-25 ms). Systolic pulmonary artery pressure was significantly higher in patients compared to controls (p=0.002); PHT was present in 28 patients (27%), while severe PHT in three (2.9%). In three patients with severe PHT, only one had impaired LV ejection fraction and increased pulmonary wedge pressure. Overall, three patients (2.9%) had a history of heart failure, two with systolic LV dysfunction, and one with severe PHT. Cardiac involvement in sickle β-thalassemia concerns biventricular dilatation and dysfunction along with PHT, leading to congestive heart failure.

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“…Available evidence now suggests that combined therapy should be the treatment of choice for patients with established cardiac failure. We have reported two cases with severe CCF who reversed with intensive combination therapy (58, 59) and we have at least eight more patients with similar outcome. Two other studies show similar responses (56, 60).…”

Section: Summary Of the Mechanisms Of Heart Injurymentioning

confidence: 90%

The heart in transfusion dependent homozygous thalassaemia today – prediction, prevention and management

Aessopos

Berdoukas

Tsironi

2007

European J of Haematology

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Cardiac disease remains the major cause of death in thalassaemia major. This review deals with the mechanisms involved in heart failure development, the peculiar clinical presentation of congestive heart failure and provides guidelines for diagnosis and management of the acute phase of cardiac failure. It emphasizes the need for intensive medical – cardiac care and aggressive iron chelating management as, with such approaches, today, the patients outcomes can be favourable in the long term. It covers advances in the assessment of cardiac iron overload with the use of magnetic resonance imaging and makes recommendations for preventing the onset of cardiac problems by tailoring iron chelation therapy appropriate to the degree of cardiac iron loading found.

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Transfusional hemosiderosis and combined chelation therapy in sickle thalassemia

Cited by 14 publications

References 14 publications

Cardiac Function and Iron Chelation in Thalassemia Major and Intermedia: A Review of the Underlying Pathophysiology and Approach to Chelation Management

Cardiac Function and Iron Chelation in Thalassemia Major and Intermedia: A Review of the Underlying Pathophysiology and Approach to Chelation Management

Cardiac involvement in sickle β-thalassemia

The heart in transfusion dependent homozygous thalassaemia today – prediction, prevention and management

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