Hereditary Disorders of Red Cell Permeability Medication

Updated: Jan 30, 2019
  • Author: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Medication

Medication Summary

The only medication used in hereditary stomatocytosis syndromes is folic acid for patients with significant hemolysis. Iron chelation with deferoxamine may be needed for patients with significant iron overload.

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Vitamins

Class Summary

These are essential for normal DNA synthesis. The only medication used to treat stomatocytosis syndromes is folic acid, which is used in patients with hemolytic anemia.

Folic acid (Folvite)

Important cofactor for enzymes required for nucleic acid synthesis and normal erythropoiesis.

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Chelating agent

Class Summary

Iron overload may occur in adults with overhydrated hereditary stomatocytosis (OHSt) and dehydrated hereditary stomatocytosis (DHSt); the mechanism is unknown.

Deferoxamine mesylate (Desferal)

Usually administered as slow SC infusion through portable pump. Freely soluble in water. Approximately 8 mg of iron is bound by 100 mg of deferoxamine. Promotes renal and hepatic excretion in urine and bile in feces. Gives urine a red discoloration. Readily chelates iron from ferritin and hemosiderin but not transferrin. Does not affect iron in cytochromes or hemoglobin. Most effective when provided to the circulation continuously by infusion. Helps prevent damage to liver and bone marrow from iron deposition. May be administered either by IM injection or by slow IV infusion. Does not effectively chelate other trace metals of nutritional importance. Provided in vials containing 500 mg of lyophilized sterile drug. 2 mL of sterile water for injection should be added to each vial, bringing the concentration to 250 mg/mL. For IV use, this may be diluted in 0.9% sterile saline, 5% dextrose solution, or Ringer solution.

IM is preferred route of administration, except in hypotension and cardiovascular collapse, in which the IV route should be considered.

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