Spherocytosis medical therapy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Treatment of Acute Symptoms

Acute symptoms of anemia and hyperbilirubinemia can indicate treatment with blood transfusions or exchanges. Transfusions treat anemia by adding healthy donor blood to the patient’s own, providing needed red blood cells. As the transfused blood does not contain elliptocytes, it will not be hemolysed per se, but the overactive spleen may still break down a significant proportion of the transfused blood. Exchanges treat hyperbilirubinemia by replacing some portion of the patient’s blood with healthy donor blood, thus removing some portion of the toxic bilirubin.

Treatment of Chronic Symptoms

Iron supplementation supports the increased production of red blood cells, but in longstanding cases in which patients have taken supplemental iron or received numerous blood transfusions, iron overload may be a significant problem, being a potential cause of cardiomyopathy and liver disease. If there is iron overload, chelation therapy with agents such as desferrioxamine may be necessary.

Treatment of the Underlying Disorder

Both measures described above treat the symptoms, not the cause of the disorder. Non-hereditary spherocytosis has several causes, each treated differently. Experimental gene therapy exists to treat hereditary spherocytosis in lab mice; however, this treatment has not yet been tried on humans and because of the risks involved in human gene therapy, it may never be. See also Hereditary spherocytosis.

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