Reversal of cardiac complications by deferiprone and deferoxamine combination therapy in a patient affected by a severe type of juvenile hemochromatosis (JH)

Gavarini, Fabio; Minonzio, Francesca; Delbini, Paola; Bianchi, Annamaria; Cappellini, Maria Domenica

doi:10.1182/blood-2006-04-016949

Cited by 80 publications

(61 citation statements)

References 16 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The man-woman ratio for median serum ferritin levels for ages 18 -45 years is 3.8, which is similar to the increased risk for heart disease, with the reduced risk against heart disease in women ending with the onset of menopause. Epidemiologic studies in overt iron overload states such as transfusional iron overload and hemochromatosis have shown that the incidence of cardiac disease is increased (64) and that treatment with iron chelation improves cardiovascular outcome (27,86,87). Similarly, several studies have demonstrated a direct association between increased iron intake, body iron stores, and cardiovascular risk in the general population.…”

Section: The Role Of Iron In Endothelial and Vascular Diseasementioning

confidence: 99%

The Role of Iron in Diabetes and Its Complications

et al. 2007

View full text Add to dashboard Cite

Section: The Role Of Iron In Endothelial and Vascular Diseasementioning

confidence: 99%

The Role of Iron in Diabetes and Its Complications

et al. 2007

View full text Add to dashboard Cite

“…A recent study by Fabio et al demonstrated that combined chelation therapy with deferoxamine and deferiprone successfully reversed the effects of heart failure in the setting of unrecognized juvenile hemochromatosis [6]. Therefore, iron chelation may prove useful in hemochromatosis cases where phlebotomy is not indicated or feasible.…”

Section: Systemic Iron Overload: Hemochromatosismentioning

confidence: 99%

Iron chelation beyond transfusion iron overload

Pietrangelo

2007

Am. J. Hematol.

View full text Add to dashboard Cite

The effects of systemic iron overload in hereditary (e.g., classic HFE hemochromatosis) or acquired disorders (e.g., transfusion-dependent iron overload) are well known. Several other iron overload diseases, with an observed mild-to-moderate increase in iron in selected organs (e.g., the liver or the brain), or with ``misdistribution{''} of iron within cells (e.g., reticuloendothelial cells) or subcellular organelles (e.g., mitochondria), have been recognized more recently. The deleterious impact of any excess iron may be high as active redox iron may directly contribute to cell damage or affect signaling pathways involved in cell necrosis-apoptosis or organ fibrosis and cancer. This article discusses the potential use of iron chelation therapy to treat iron overload from causes other than transfusion overload.

show abstract

“…We recommend the following management strategy: ·d −1 (the total dose given in 3 divided doses). 50,[221][222][223][224][225][226] 5. Supportive hemodynamic therapy should be geared to maintain cerebral and renal perfusion, avoiding aggressive inotropic therapy, which can be detrimental.…”

Section: Management Of Acute Decompensated Hf In Tmmentioning

confidence: 99%

Cardiovascular Function and Treatment in β-Thalassemia Major

Pennell¹,

Udelson²,

Arai³

et al. 2013

Circulation

332

172

View full text Add to dashboard Cite

This aim of this statement is to report an expert consensus on the diagnosis and treatment of cardiac dysfunction in β-thalassemia major (TM). This consensus statement does not cover other hemoglobinopathies, including thalassemia intermedia and sickle cell anemia, in which a different spectrum of cardiovascular complications is typical. There are considerable uncertainties in this field, with a few randomized controlled trials relating to treatment of chronic myocardial siderosis but none relating to treatment of acute heart failure. The principles of diagnosis and treatment of cardiac iron loading in TM are directly relevant to other iron-overload conditions, including in particular Diamond-Blackfan anemia, sideroblastic anemia, and hereditary hemochromatosis. Heart failure is the most common cause of death in TM and primarily results from cardiac iron accumulation. The diagnosis of ventricular dysfunction in TM patients differs from that in nonanemic patients because of the cardiovascular adaptation to chronic anemia in non–cardiac-loaded TM patients, which includes resting tachycardia, low blood pressure, enlarged end-diastolic volume, high ejection fraction, and high cardiac output. Chronic anemia also leads to background symptomatology such as dyspnea, which can mask the clinical diagnosis of cardiac dysfunction. Central to early identification of cardiac iron overload in TM is the estimation of cardiac iron by cardiac T2* magnetic resonance. Cardiac T2* <10 ms is the most important predictor of development of heart failure. Serum ferritin and liver iron concentration are not adequate surrogates for cardiac iron measurement. Assessment of cardiac function by noninvasive techniques can also be valuable clinically, but serial measurements to establish trends are usually required because interpretation of single absolute values is complicated by the abnormal cardiovascular hemodynamics in TM and measurement imprecision. Acute decompensated heart failure is a medical emergency and requires urgent consultation with a center with expertise in its management. The first principle of management of acute heart failure is control of cardiac toxicity related to free iron by urgent commencement of a continuous, uninterrupted infusion of high-dose intravenous deferoxamine, augmented by oral deferiprone. Considerable care is required to not exacerbate cardiovascular problems from overuse of diuretics or inotropes because of the unusual loading conditions in TM. The current knowledge on the efficacy of removal of cardiac iron by the 3 commercially available iron chelators is summarized for cardiac iron overload without overt cardiac dysfunction. Evidence from well-conducted randomized controlled trials shows superior efficacy of deferiprone versus deferoxamine, the superiority of combined deferiprone with deferoxamine versus deferoxamine alone, and the equivalence of deferasirox versus deferoxamine.

show abstract

Reversal of cardiac complications by deferiprone and deferoxamine combination therapy in a patient affected by a severe type of juvenile hemochromatosis (JH)

Cited by 80 publications

References 16 publications

The Role of Iron in Diabetes and Its Complications

The Role of Iron in Diabetes and Its Complications

Iron chelation beyond transfusion iron overload

Cardiovascular Function and Treatment in β-Thalassemia Major

Contact Info

Product

Resources

About