The success of chelation therapy in controlling iron overload in patients with thalassemia major is highly variable and may partly depend on the rate of transfusional iron loading. Using data from the 1-year phase III study of deferasirox, including volumes of transfused red blood cells and changes in liver iron concentration (LIC) in 541 patients, the effect of iron loading on achieving neutra...

We describe a new high performance liquid chromatography coupled with ultraviolet detection method for the quantification of plasma concentration of oral iron chelating agent deferasirox. A simple protein precipitation extraction procedure was applied on 500 μl of plasma aliquots. Chromatographic separation was achieved on a C18 reverse phase column and eluate was monitored at 295 nm, with 8 mi...

AIM Treatment of chronic kidney disease (CKD) includes parenteral iron therapy, and these infusions can lead to iron overload. Secondary iron overload is typically treated with iron chelators, of which deferasirox is one of the most promising. However, it has not been studied in patients with CKD and iron overload. METHODS A pilot study was conducted to evaluate the pharmacokinetics and safet...

Transfusion-associated iron overload induces systemic toxicity. Deferasirox, a convenient long acting oral agent, has recently been introduced in clinical practice with a promising efficacy. But there are some patients who experience drug-related toxicities and cannot tolerate it. To investigate effect of genetic variations on the toxicities and find optimal target population, we analyzed the g...

Treatment of iron overload using deferoxamine (DFO) is associated with significant deficits in patients' health-related quality of life (HRQOL) and low treatment satisfaction. The current article presents patient-reported HRQOL, satisfaction, adherence, and persistence data from β-thalassemia (n = 274) and myelodysplastic syndrome (MDS) patients (n = 168) patients participating in the Evaluatio...

Juvenile hemochromatosis (JH) is a rare condition classified as an autosomal recessive disorder that leads to severe iron absorption. JH usually affects people under the age of 30 and presents symptoms such as chronic liver damage, hypogonadotropic hypogonadism, cardiac diseases and endocrine dysfunctions. The present case reports a 29-year-old Brazilian woman with JH condition due to HAMP muta...

Background: Invasive Fungal Sinusitis(IFS) is seen in both immunocompromised and immunocompetent patients. All fungi elaborate uptake mechanisms to sequester iron, produce siderophores with higher affinity for iron. The availability of iron ability utilize them are essential viability growth Mucor Aspergillus. Currently, IFS dealt surgical debridement aggressive medical therapy. Amphotericin B ...

We read with interest the article by Pepe et al. wherein the authors retrospectively compared cardiac iron burden measured by T2* magnetic resonance imaging (MRI) in three groups of beta-thalassemia major patients treated with desferrioxamine, deferiprone, or deferasirox monotherapy for more than one year. The mean global heart T2* value was significantly higher in the deferiprone compared to t...

A 59-year-old man presented with refractory anemia, choreoathetosis in the left upper extremity, an unsteady gait and cognitive dysfunction. The laboratory findings showed a marked decrease in ceruloplasmin. Magnetic resonance images revealed iron deposition in the brain and visceral organs. Iron accumulation was also observed in hepatocytes. Genetic analyses of the ceruloplasmin gene revealed ...

To the Editor: Improvements in managing b-thalassaemia major have allowed many patients to survive beyond puberty. Fertility can, however, be impaired as a result of iron overload-related hypogonadism (1), although assisted reproductive techniques and advances in treating iron overload have increased the number of successful pregnancies in such patients (2–9). During pregnancy, patients may req...