Hereditary spherocytosis; Spherocytosis

Surgery to remove the spleen (splenectomy) cures the anemia but doesn't correct the abnormal cell shape.

Families with a history of spherocytosis should have their children screened for this disorder.

Children should wait until age 5 to have splenectomy because of the infection risk. In mild cases discovered in adults, it may not be necessary to remove the spleen.

Children and adults should be given a pneumococcal vaccine before spleen removal surgery, and also may receive folic acid supplements. Additional vaccines may be indicated based on the patient’s history.

This outcome is usually good with treatment. After the spleen is removed, the life span of the red blood cell returns to normal.

• Gallstones
• Much lower red blood cell production (aplastic crisis) caused by a viral infection, which can make anemia worse

Call your health care provider for an appointment if your symptoms get worse, do not improve with treatment, or if you develop new symptoms.

Images

Red blood cells, normal

Blood cells

Red blood cells, spherocytosis

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Review Date: 03/28/2010
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and James R. Mason, MD, Oncologist, Director, Blood and Marrow Transplantation Program and Stem Cell Processing Lab, Scripps Clinic, Torrey Pines, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org)