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Last update: 10 - 03 - 2008

 Ovalocytosis hereditary    

Ovalocytosis hereditary

Hereditary ovalocytosis is an inherited condition in which the red blood cells (RBCs) have a slightly oval or elliptical shape. Also known as hereditary elliptocytosis. This condition is associated with mild haemolytic anaemia. In severe cases, the disease may be treated by removal of the spleen  (splenectomy). Southeast Asian ovalocytosis is a special type of ovalocytosis. Erythrocytes in Southeast Asian ovalocytosis have a recognizable shape and are poorly deformable, due to a deletion of nine amino acids in Band 3, a transmembrane glycoprotein involved in ions transport. The phenotype is autosomal dominant, occurs only in the heterozygous state, and is asymptomatic.

Synonym: Hereditary elliptocytosis

   
  

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