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Hereditary Elliptocytosis Treatment & Management

  • Author: Daniel J Kim, MD, MS; Chief Editor: Emmanuel C Besa, MD  more...
Updated: Feb 09, 2016

Medical Care

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.


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, 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.

Contributor Information and Disclosures

Daniel J Kim, MD, MS Staff Physician, Department of Medicine, Olive View-UCLA Medical Center

Daniel J Kim, MD, MS is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Medical Association, American Society of Hematology, California Medical Association, Christian Medical and Dental Associations, American Society of Clinical Oncology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Marcel E Conrad, MD Distinguished Professor of Medicine (Retired), University of South Alabama College of Medicine

Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, SWOG

Disclosure: Partner received none from No financial interests for none.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Karen Seiter, MD Professor, Department of Internal Medicine, Division of Oncology/Hematology, New York Medical College

Karen Seiter, MD is a member of the following medical societies: American Association for Cancer Research, American College of Physicians, American Society of Hematology

Disclosure: Received honoraria from Novartis for speaking and teaching; Received consulting fee from Novartis for speaking and teaching; Received honoraria from Celgene for speaking and teaching.


Leland D Powell, MD, PhD Associate Clinical Professor of Medicine, David Geffen School of Medicine at UCLA; Consulting Staff, Department of Medicine, Olive View-UCLA Medical Center

Disclosure: Nothing to disclose.

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