MRI T2* is essential for people with transfusional iron overload
Learn more about why and when to get it done.
In patients with transfusion-related iron overload, the two key target organs for iron accumulation are the heart and liver. Although the liver is the major site of iron storage, the smaller amount of iron that goes to the heart can have more serious clinical consequences, including arrhythmias, cardiac failure, and death.
Since serum ferritin and liver iron are not strong predictors of the level of iron deposition in the heart, cardiac iron content should be directly evaluated on a regular basis in patients with transfusion-dependent thalassemia syndromes, in order to determine the risk of iron-related cardiac damage and monitor the effectiveness of the patient’s current chelation regimen in removing excess iron from the heart.
Magnetic resonance imaging (MRI) is currently the preferred method for assessing iron levels in the heart, as it is non-invasive, widely available, and able to measure iron load in a reliable and quantitative manner. The recommended MRI sequence for measuring cardiac iron is T2*, which is inversely correlated with iron content, i.e., the lower the value, the higher the iron level.
Cardiac T2* values 20 milliseconds (ms) or lower indicate clinically significant levels of cardiac iron loading.
Assessment of cardiac iron by MRI T2* should start early in the patient’s life, ideally around age 6 to 10. If an older patient has never previously been evaluated using cardiac MRI, he or she should undergo a baseline scan as soon as possible to help determine his or her risk profile and define the optimal strategy for chelation therapy.
The recommended frequency of ongoing cardiac MRI monitoring will depend on the patient’s degree of cardiac iron overload:
If a patient’s initial scan(s) show(s) heart iron in the normal range, (above 20 ms), follow-up scans should be performed approximately annually thereafter.
Scans should be completed more frequently – every 6 months – in patients at higher cardiac risk due to elevated cardiac iron content (T2* under 10 ms), poor treatment compliance, or reduced left ventricular ejection fraction.
The cardiac T2* sequence can be run on most MRI machines currently in use, provided that the appropriate software has been installed. To find the center nearest you that offers cardiac T2*, enter your address and/or ZIP code into the interactive map.
Centers were identified based on their capacity to perform cardiac T2* monitoring. For your convenience we have also specified the liver imaging services offered by these sites, but it should be noted that this map does not represent a complete listing of centers offering liver iron assessment.
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IMPORTANT SAFETY INFORMATION
Indication
Ferriprox® (deferiprone) is an iron chelator indicated for the treatment of transfusional iron overload in patients with:
- thalassemia syndromes
- sickle cell disease or other anemias
Ferriprox Tablets are indicated in adult and pediatric patients ≥8 years of age; Ferriprox Oral Solution is indicated in patients ≥3 years of age.
Limitations of Use:
Safety and effectiveness have not been established for the treatment of transfusional iron overload in patients with myelodysplastic syndrome or in patients with Diamond Blackfan anemia.
WARNING: AGRANULOCYTOSIS AND NEUTROPENIA
- Ferriprox can cause agranulocytosis that can lead to serious infections and death. Neutropenia may precede the development of agranulocytosis.
- Measure the absolute neutrophil count (ANC) before starting Ferriprox and monitor regularly while on therapy.
- Interrupt Ferriprox therapy if neutropenia develops.
- Interrupt Ferriprox if infection develops, and monitor the ANC more frequently.
- Advise patients taking Ferriprox to report immediately any symptoms indicative of infection.
Indication
Ferriprox® (deferiprone) is an iron chelator indicated for the treatment of transfusional iron overload in patients with:1
- thalassemia syndromes
- sickle cell disease or other anemias
Ferriprox Tablets are indicated in adult and pediatric patients ≥8 years of age; Ferriprox Oral Solution is indicated in patients ≥3 years of age.
Limitations of Use:
Safety and effectiveness have not been established for the treatment of transfusional iron overload in patients with myelodysplastic syndrome or in patients with Diamond Blackfan anemia.
Important Safety Information
Ferriprox is contraindicated in patients with known hypersensitivity to deferiprone or to any of the excipients in the formulations.
In pooled clinical trials, 7.5% of 642 patients with thalassemia syndromes treated with Ferriprox developed increased ALT values. Four (0.62%) Ferriprox-treated subjects discontinued the drug due to increased serum ALT levels and 1 (0.16%) due to an increase in both ALT and AST. In pooled clinical trials, 7.7% of 196 patients with sickle cell disease or other anemias treated with Ferriprox developed increased ALT values. Monitor serum ALT values monthly during therapy with Ferriprox and consider interruption of therapy if there is a persistent increase in the serum transaminase levels. Decreased plasma zinc concentrations have been observed on deferiprone therapy. Monitor plasma zinc annually, and supplement in the event of a deficiency.
Ferriprox can cause fetal harm. Advise females of reproductive potential to use an effective method of contraception during treatment with Ferriprox and for at least six months after the last dose. Advise males with female partners of reproductive potential to use effective contraception during treatment with Ferriprox and for at least three months after the last dose. Advise females not to breastfeed during treatment with Ferriprox and for at least 2 weeks after the last dose.
Avoid co-administration of Ferriprox with other drugs known to be associated with neutropenia or agranulocytosis; however, if this is unavoidable, closely monitor the absolute neutrophil count. Avoid co-administration with UGT1A6 inhibitors. Allow at least a 4-hour interval between administration of Ferriprox and drugs or supplements containing polyvalent cations (e.g., iron, aluminum, or zinc).
The most common adverse reactions in patients with thalassemia (incidence ≥6%) are nausea, vomiting, abdominal pain, arthralgia, ALT increased and neutropenia. The most common adverse reactions in patients with sickle cell disease or other anemias (incidence ≥6%) are pyrexia, abdominal pain, bone pain, headache, vomiting, pain in extremity, sickle cell anemia with crisis, back pain, ALT increased, AST increased, arthralgia, oropharyngeal pain, nasopharyngitis, neutrophil count decreased, cough and nausea.
Inform patients that their urine might show a reddish/brown discoloration due to the excretion of the iron-deferiprone complex. This is a very common sign of the desired effect, and it is not harmful.
Advise patients to avoid alcohol while taking Ferriprox tablets (twice-a-day). Consumption of alcohol while taking Ferriprox tablets (twice-a-day) may result in more rapid release of deferiprone.
Please see Full Prescribing Information, including boxed WARNING, and Medication Guide.
IMPORTANT SAFETY INFORMATION
Indication
Ferriprox® (deferiprone) is an iron chelator indicated for the treatment of transfusional iron overload in patients with:
- thalassemia syndromes
- sickle cell disease or other anemias
Ferriprox Tablets are indicated in adult and pediatric patients ≥8 years of age; Ferriprox Oral Solution is indicated in patients ≥3 years of age.
Limitations of Use:
Safety and effectiveness have not been established for the treatment of transfusional iron overload in patients with myelodysplastic syndrome or in patients with Diamond Blackfan anemia.
WARNING: AGRANULOCYTOSIS AND NEUTROPENIA
- Ferriprox can cause agranulocytosis that can lead to serious infections and death. Neutropenia may precede the development of agranulocytosis.
- Measure the absolute neutrophil count (ANC) before starting Ferriprox and monitor regularly while on therapy.
- Interrupt Ferriprox therapy if neutropenia develops.
- Interrupt Ferriprox if infection develops, and monitor the ANC more frequently.
- Advise patients taking Ferriprox to report immediately any symptoms indicative of infection.
Indication
Ferriprox® (deferiprone) is an iron chelator indicated for the treatment of transfusional iron overload in patients with:1
- thalassemia syndromes
- sickle cell disease or other anemias
Ferriprox Tablets are indicated in adult and pediatric patients ≥8 years of age; Ferriprox Oral Solution is indicated in patients ≥3 years of age.
Limitations of Use:
Safety and effectiveness have not been established for the treatment of transfusional iron overload in patients with myelodysplastic syndrome or in patients with Diamond Blackfan anemia.
Important Safety Information
Ferriprox is contraindicated in patients with known hypersensitivity to deferiprone or to any of the excipients in the formulations.
In pooled clinical trials, 7.5% of 642 patients with thalassemia syndromes treated with Ferriprox developed increased ALT values. Four (0.62%) Ferriprox-treated subjects discontinued the drug due to increased serum ALT levels and 1 (0.16%) due to an increase in both ALT and AST. In pooled clinical trials, 7.7% of 196 patients with sickle cell disease or other anemias treated with Ferriprox developed increased ALT values. Monitor serum ALT values monthly during therapy with Ferriprox and consider interruption of therapy if there is a persistent increase in the serum transaminase levels. Decreased plasma zinc concentrations have been observed on deferiprone therapy. Monitor plasma zinc annually, and supplement in the event of a deficiency.
Ferriprox can cause fetal harm. Advise females of reproductive potential to use an effective method of contraception during treatment with Ferriprox and for at least six months after the last dose. Advise males with female partners of reproductive potential to use effective contraception during treatment with Ferriprox and for at least three months after the last dose. Advise females not to breastfeed during treatment with Ferriprox and for at least 2 weeks after the last dose.
Avoid co-administration of Ferriprox with other drugs known to be associated with neutropenia or agranulocytosis; however, if this is unavoidable, closely monitor the absolute neutrophil count. Avoid co-administration with UGT1A6 inhibitors. Allow at least a 4-hour interval between administration of Ferriprox and drugs or supplements containing polyvalent cations (e.g., iron, aluminum, or zinc).
The most common adverse reactions in patients with thalassemia (incidence ≥6%) are nausea, vomiting, abdominal pain, arthralgia, ALT increased and neutropenia. The most common adverse reactions in patients with sickle cell disease or other anemias (incidence ≥6%) are pyrexia, abdominal pain, bone pain, headache, vomiting, pain in extremity, sickle cell anemia with crisis, back pain, ALT increased, AST increased, arthralgia, oropharyngeal pain, nasopharyngitis, neutrophil count decreased, cough and nausea.
Inform patients that their urine might show a reddish/brown discoloration due to the excretion of the iron-deferiprone complex. This is a very common sign of the desired effect, and it is not harmful.
Advise patients to avoid alcohol while taking Ferriprox tablets (twice-a-day). Consumption of alcohol while taking Ferriprox tablets (twice-a-day) may result in more rapid release of deferiprone.
Please see Full Prescribing Information, including boxed WARNING, and Medication Guide.