Stephen J. Schueler, M.D.

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Hereditary Spherocytosis Overview

Another name for Hereditary Spherocytosis is Spherocytosis.

What is spherocytosis?
A person with spherocytosis has an inherited disease that results in abnormally shaped red blood cells. The red blood cells are round, rather than elliptical. The deformed red blood cells have a shorter lifespan than normal red blood cells. This leads to a shortage of red blood cells in the bloodstream, called hemolytic anemia.

What are the symptoms of spherocytosis?
Symptoms of spherocytosis are the same as those of hemolytic anemia. Initial symptoms of spherocytosis may include anorexia, weakness, fatigue, pale skin, headaches, dizziness, and faintness upon standing. Symptoms of worsening spherocytosis may include chest pain, difficulty breathing with exertion, fainting, palpitations, jaundice, and brown urine.

How does the doctor treat spherocytosis?
Treatment for spherocytosis may include blood transfusions, iron supplements, vitamin B supplements, and surgery to remove the spleen.

Continue to Hereditary Spherocytosis Risk Factors

Last Updated: Sep 30, 2010 References
Authors: Stephen J. Schueler, MD; John H. Beckett, MD; D. Scott Gettings, MD
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PubMed Hereditary Spherocytosis References
  1. Dhaliwal G, Cornett PA, Tierney LM Jr. Hemolytic anemia. Am Fam Physician. 2004 Jun 1;69(11):2599-606. [15202694]
  2. Perkins SL. Pediatric red cell disorders and pure red cell aplasia. Am J Clin Pathol. 2004 Dec;122 Suppl:S70-86. [15690644]
  3. Stoehr GA, Luecken J, Zielen S, Eber SW, Borrow R, Rose MA. Mode of splenectomy and immunogenicity of meningococcal vaccination in patients with hereditary spherocytosis. Br J Surg. 2008 Apr;95(4):466-71. [18161897]
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