Congenital spherocytic anemia

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Blood cells

Red blood cells, normal

Red blood cells, spherocytosis

Hereditary spherocytosis; Spherocytosis

Splenectomy (surgical removal of the spleen) cures the anemia of spherocytosis. Although the abnormal cell defect persists, the red cell life span returns to normal.

Families with a history of spherocytosis should have their children screened for this disorder. In mild cases discovered in adult life, splenectomy may not be necessary.

Splenectomy should be delayed in children until the age of 5 to prevent unnecessary infection. Pneumococcal immunization (vaccine) is given prior to the surgery. Folic acid supplementation can be given to children.

This outcome is usually good with treatment.

• Development of gallstones from the pigment derived from the destroyed red blood cells
• Aplastic crises (severe decrease in red blood cell production) caused by a viral infection

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

Review Date: 07/29/2005
Reviewed By: William Matsui, M.D., Assistant Professor of Oncology, Division of Hematologic Malignancies, The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Baltimore, MD. Review provided by VeriMed Healthcare Network.