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Patients
with aplastic anemia, beta-thalassemia and myelodysplastic syndrome who
are on repeated blood transfusion are known to have an increased iron
content in their body. This condition is known as transfusion
hemosiderosis. The presence of high amount of iron in the body can
lead to damage of the liver, heart, and endocrine glands,
resulting
in organ compromise andeventually death.
Normal body iron stores in the typical patient are 3– 4 g; an excess of iron of 20 g or more can lead to organ damage. Each unit of transfused red blood cells averages 200–250 mg of iron.
Measurement of serum ferritin is the most commonly used indirect estimate of body iron stores. This is not a reliable marker of hepatic or liver iron burden. Ferritin levels are affected by infection, inflammation, liver disease, vitamin C deficiency, and hemolysis. The only way to remove iron from the body is to use iron chelators.
Deferoxamine and defereprone have been used in iron chelation in these situations.
Deferoxamine has a 1:1 iron molecule binding ratio makes it an effective agent for the chelation of excess iron. The major draw back with deferoxamine is the fact owing to its shorter half life and lack of oral bioavailability, the drug has to be to be given as a subcutaneous infusion over 8–12 hours, 5–7 days per week.
Defereprone is an oral iron chelator, This drug had the draw back of casuing agranulocytosis.
A new drug named Deferasirox, an oral iron chelator has been given approval by the FDA for the treatment of chronic iron overload due to blood transfusions in patients 2 years of age and older.
The drug is marketed as Exjade. In India two versions are availabe one marketed by Cipla as Desirox
and the other marketed by Novartis as Asunra.
Deferasirox is an oral once daily iron chelator, approved by the FDA for treatment of transfusional
chronic iron overloead. Two molecules of deferasirox binds to one Fe3+ . The normal dosage is 20-30 mg /kg body weight. ( Please contact your doctor to calculate the actual dosage, which may depend on your serrum ferritin levels, serum creatinine and liver function tests.)
The most common advesre event associated with deferasirox includenausea, vomiting, abdominal pain, constipation , diarrhoea and skin rash.
It may also elevate serum creatinine levels, ALT and may cause visual and audior disturbances. . Regular monitoring of serum creatinine and liver function tests are recommended in patients on deferasirox.
Further reading:
1. Yang LPH, Keam SJ, Keating GM. Deferasirox. A review of its use in the management of transfusional chronic iron overlaod. Drugs. 2007; 67: 2211-2230.
2. Cappellini MD et al. A phaze 3 study of deferasirox (ICL 670), a once daily iron chelator, in patients with Beta thalessemia. Blood 2006; 107: 3455-3462.
3. VanOrden HE and Hageman TM. Deferasirox-an oral agent for chronic iron overload. Ann Pharmcother 2006; 40: 1110-7.
4. Stumpf JL. Deferasirox Am J Health Syst Pharm 2007; 64: 606-16.
Normal body iron stores in the typical patient are 3– 4 g; an excess of iron of 20 g or more can lead to organ damage. Each unit of transfused red blood cells averages 200–250 mg of iron.
Measurement of serum ferritin is the most commonly used indirect estimate of body iron stores. This is not a reliable marker of hepatic or liver iron burden. Ferritin levels are affected by infection, inflammation, liver disease, vitamin C deficiency, and hemolysis. The only way to remove iron from the body is to use iron chelators.
Deferoxamine and defereprone have been used in iron chelation in these situations.
Deferoxamine has a 1:1 iron molecule binding ratio makes it an effective agent for the chelation of excess iron. The major draw back with deferoxamine is the fact owing to its shorter half life and lack of oral bioavailability, the drug has to be to be given as a subcutaneous infusion over 8–12 hours, 5–7 days per week.
Defereprone is an oral iron chelator, This drug had the draw back of casuing agranulocytosis.
A new drug named Deferasirox, an oral iron chelator has been given approval by the FDA for the treatment of chronic iron overload due to blood transfusions in patients 2 years of age and older.
The drug is marketed as Exjade. In India two versions are availabe one marketed by Cipla as Desirox
and the other marketed by Novartis as Asunra.
Deferasirox is an oral once daily iron chelator, approved by the FDA for treatment of transfusional
chronic iron overloead. Two molecules of deferasirox binds to one Fe3+ . The normal dosage is 20-30 mg /kg body weight. ( Please contact your doctor to calculate the actual dosage, which may depend on your serrum ferritin levels, serum creatinine and liver function tests.)
The most common advesre event associated with deferasirox includenausea, vomiting, abdominal pain, constipation , diarrhoea and skin rash.
It may also elevate serum creatinine levels, ALT and may cause visual and audior disturbances. . Regular monitoring of serum creatinine and liver function tests are recommended in patients on deferasirox.
Further reading:
1. Yang LPH, Keam SJ, Keating GM. Deferasirox. A review of its use in the management of transfusional chronic iron overlaod. Drugs. 2007; 67: 2211-2230.
2. Cappellini MD et al. A phaze 3 study of deferasirox (ICL 670), a once daily iron chelator, in patients with Beta thalessemia. Blood 2006; 107: 3455-3462.
3. VanOrden HE and Hageman TM. Deferasirox-an oral agent for chronic iron overload. Ann Pharmcother 2006; 40: 1110-7.
4. Stumpf JL. Deferasirox Am J Health Syst Pharm 2007; 64: 606-16.
Last
updated on 6 September 2008