Hereditary Elliptocytosis Treatment & Management
- Author: Daniel J Kim, MD, MS; Chief Editor: Emmanuel C Besa, MD more...
Most patients with hereditary elliptocytosis (HE) do not require medical treatment. A diet rich in folic acid or folic acid supplementation is recommended to avoid consequences of folate deficiency in a hemolytic state. Other supportive measures, such as blood transfusions, may be indicated if the anemia is severe.
Because the spleen is the site for erythrocyte destruction, splenectomy markedly improves anemia for patients with clinically significant hemolysis. Splenectomy stops or markedly reduces hemolysis that results from HE but does not correct the underlying membrane defect. As with splenectomy for other indications, the pneumococcal, meningococcal, and Haemophilus influenzae vaccines should be administered before surgery.
Consultation with a general surgeon is indicated if splenectomy is being considered in a patient with clinically significant uncompensated hemolysis. Consultation with a genetic counselor is helpful to explain the genetic nature and implications of this disease to immediate family members.
A diet with adequate folic acid (green leafy vegetables) or folic acid supplements is advisable to prevent folate deficiency.
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