Hereditary Spherocytosis

Hereditary Spherocytosis is a rare inherited disorder, where the normally disc-shaped red blood cells become spherical. The rigid, misshapen red blood cell is trapped and destroyed in the person’s spleen, causing enlarged spleenHereditary Spherocytosis and anemia.

The anemia is generally mild, but can become more severe if an infection develops, when the Hereditary Spherocytosis is severe; anemia and jaundice can progress, the gallstones can form, and liver can enlarge. In young individuals, the hereditary spherocytosis can be mistaken for hepatitis.

Abnormalities of bone, such as extra toes and fingers, and a tower-shaped skull, may occur. Treatment for Hereditary Spherocytosis usually is not required, but severe anemia can require spleen removal. Such procedure does not correct red blood cell shape, but it reduces the number that is destroyed and thus corrects the anemia.

How Hereditary Spherocytosis Causes Anemia

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