hereditary spherocytosis

spherocytosis /sphe·ro·cy·to·sis/ (sfēr″o-si-to´sis) the presence of spherocytes in the blood.

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hereditary spherocytosis  a congenital hereditary form of hemolytic anemia characterized by spherocytosis, abnormal fragility of erythrocytes, jaundice, and splenomegaly.

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hereditary spherocytosis

n.

A congenital defect in the cell membrane of red blood cells resulting in thickened, fragile red blood cells that are susceptible to spontaneous hemolysis and marked by chronic anemia, reticulocytosis, mild jaundice, and fever and abdominal pain. Also called chronic acholuric jaundice, chronic familial jaundice, congenital hemolytic anemia, congenital hemolytic jaundice, spherocytic anemia.

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Hereditary spherocytosis (HS)

A blood disorder in which the red blood cells are relatively fragile and are damaged or destroyed when they pass through the spleen. Splenectomy is the only treatment for HS.

Mentioned in: Splenectomy

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hereditary spherocytosis.

See spherocytic anemia.

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hereditary spherocytosis

Spherocytosis Neonatology An AD condition characterized by ↑ osmotic fragility of red cells, autohemolysis, splenomegaly and mild anemia partially corrected by splenectomy; HS is linked to a defect in a 28 kD RBC membrane protein Management Splenectomy. Cf Spherocytosis.