Hereditary spherocytosis

From Health Encyclopedia

Contents 1 Used for 2 Broader Terms 3 Related Terms 4 Scope Note 5 Facts (generated by robot; please edit if you find it inaccurate)

Used for

Minkowski Chauffard syndrome

Broader Terms

congenital hemolytic anemia, genetic disorder

Related Terms

autosomal dominant trait

Scope Note

autosomal dominant trait marked by splenomegaly, jaundice, and fragile, spheroid erythrocytes which are prematurely cleared by the spleen; anemia is usually mild; treatment is splenectomy.

Facts (generated by robot; please edit if you find it inaccurate)

• Hereditary spherocytosis is most common in people of northern European ancestry.
• Hereditary spherocytosis is the most common red blood cell membrane disorder and often is associated with hemolytic crisis and premature cholelithiasis.
• Hereditary spherocytosis is a congenital haemolytic anaemia due to defect in spectrin-a RBC membrane protein and is transmitted as autosomal dominant.
• Disease: Hereditary spherocytosis Synonyms: HS Spherocytosis, severe atypical, due to suspected ankyrin defect Information about Hereditary spherocytosis is available from: The Online Mendelian Inheritance in Man (OMIM) database contains genetics resources that discuss Hereditary spherocytosis.
• Hereditary spherocytosis is a relatively common haematological disorder and will be encountered by all haematologists.

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