Alpha Thalassemia Medication

Updated: Oct 16, 2018
  • Author: Alexandra C Cheerva, MD, MS; Chief Editor: Hassan M Yaish, MD  more...
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Medication

Medication Summary

No medications are needed for silent carriers or individuals with alpha thalassemia trait. In general, no medications are needed for patients with hemoglobin H (HbH) disease; however, if the reticulocyte count is elevated, the diet should be supplemented with folic acid. If a patient has an elevated ferritin level, chelation therapy with deferoxamine or deferasirox should be considered. Deferasirox is preferred because it is orally administered, whereas deferoxamine is administered intravenously (IV) or subcutaneously (SC).

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Vitamins

Class Summary

This agent is a supplement with folic acid, a vitamin necessary for red blood cell (RBC) production.

Folic acid (Folacin-800)

Folic acid is a necessary coenzyme for nucleoprotein synthesis and maintenance in patients with erythropoiesis.

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Chelation agents

Class Summary

Iron overload (usually from multiple transfusions) may require chelation therapy, which usually begins when the ferritin level is greater than 1000 ng/mL.

Deferoxamine mesylate (Desferal)

Deferoxamine is freely soluble in water. Approximately 8 mg of iron is bound by 100 mg of deferoxamine. Deferoxamine is excreted in urine and bile and discolors the urine red. It readily chelates iron from ferritin and hemosiderin but not from transferrin. It is most effective when provided to the circulation continuously by means of infusion. Deferoxamine may be administered by intramuscular (IM) injection, slow infusion, SC bolus, or continuous infusion. It does not effectively chelate other trace metals of nutritional importance.

Deferasirox (Exjade, Jadenu)

Deferasirox is available as a tablet for oral suspension. It is an oral iron-chelating agent that reduces liver iron concentration and serum ferritin levels. Deferasirox binds iron with high affinity in a 2:1 ratio. It is approved for treatment of treat chronic iron overload due to multiple blood transfusions and nontransfusion-dependent thalassemia.

Jadenu is a newer form of Exjade that is supposed to be easier to administer and better tolerated. The dose is usually 30% lower than Exjade rounded to the nearist whole tablet.

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