Effect of age at the start of iron chelation therapy on gonadal function in beta-thalassemia major

Article Abstract:

As survival rates for people with thalassemia major (a disorder of hemoglobin, the substance that carries oxygen and iron in the blood) have improved, clinical concern has turned to the problems of growth, sexual development, and fertility faced by these patients. Formerly, few people who suffered from thalassemia major progressed normally through puberty. However, treatment with deferoxamine (a drug that chelates iron, allowing it to reduce the body's iron levels) and blood transfusions has allowed many patients to develop normally. To evaluate the effect of long-term treatment of this disorder, 40 transfusion-dependent patients were studied. Prior to the age of 10, patients with thalassemia major usually grow normally, with delayed growth occurring at later ages. Therefore, the study group included 19 patients who had begun deferoxamine treatment before the age of 10; the average age was seven and one half years (group A). The second group consisted of 21 patients who began treatment after 10 years of age; the average age was 14 years (group B). Patients were evaluated approximately 10 years after they had started treatment. Their height, weight, physical condition, bone age, and blood levels of several factors were measured. Results showed that group A patients had received more deferoxamine than group B patients, and that compliance with the treatment routine declined among patients aged 10 to 20. Ninety percent of group A patients attained puberty normally, compared with only thirty-eight percent of group B patients. Patients with abnormal puberty tended to be older when deferoxamine treatment began, and to have higher ferritin (the stored form of iron) before and during treatment. Overall, the results support early deferoxamine therapy for children with thalassemia major to maximize the chances for normal growth and sexual development. (Consumer Summary produced by Reliance Medical Information, Inc.)

Measurement, Complications and side effects, Demographic aspects, Growth, Growth (Physiology), Ferritin, Chelation therapy

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Iron-chelation therapy with oral deferiprone in patients with thalassemia major

Article Abstract:

The chelating drug deferiprone may be effective in reducing excessive iron levels in the body from repeated blood transfusions used to treat thalassemia major. Thalassemia is an anemia caused by abnormal hemoglobin synthesis. Twenty-one patients with thalassemia major took 75 milligrams of deferiprone per kilogram of body weight every day for one to five years. Ten patients had iron levels high enough to damage the liver and heart. Deferiprone caused a drop in body iron below toxic levels in eight of these patients. These 10 patients had failed therapy with another chelating drug, deferoxamine. The remaining 11 patients had been successfully treated with deferoxamine, but were switched to deferiprone because it is easier to take. Deferiprone maintained their iron levels below the toxic level. Three patients were not totally compliant with treatment, but even they responded to the drug. Side effects were minimal, but the drug is still considered investigational.

Chelating agents, Blood transfusion

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The beta-thalassemias

Article Abstract:

Beta-thalassemias are congenital anemia caused by mutations or deletions in genes encoding the synthesis of the beta-globin components of hemoglobin. Patients with the condition may have red-blood-cell synthesis rates 10 times normal levels, yet produce ineffective cells and develop iron overload. Iron accumulation in organs and tissues causes widespread damage, and patients may require both regular blood transfusions and iron chelating drugs to eliminate excess iron. Bone marrow transplantation can be highly effective.

United States, Deferoxamine

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