Hereditary Elliptocytosis Treatment & Management

Updated: Nov 06, 2019
  • Author: Daniel J Kim, MD, MS; Chief Editor: Emmanuel C Besa, MD  more...
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Treatment

Approach Considerations

In most cases, hereditary elliptocytosis (HE) causes no symptoms and requires no therapy. For patients with clinically significant hemolytic anemia, splenectomy provides marked improvement. 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.

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Surgical Care

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, pneumococcal, meningococcal, and Haemophilus influenzae vaccines should be administered before surgery.

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Consultations

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.

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