Hereditary elliptocytosis epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Epidemiology and Demographics

• The incidence of hereditary elliptocytosis is hard to determine, as many sufferers of the milder forms of the disorder are asymptomatic and their condition never comes to medical attention.
• Around 90% of those with this disorder are thought to fall into the asymptomatic population.

Developed Countries

• It is estimated that its incidence is between 3 and 5 per 10,000 in the USA

Developing Countries

• Those of African and Mediterranean descent are of higher risk.
• Some subtypes of hereditary elliptocytosis are significantly more prevalent in regions where malaria is endemic.
• For example, in equatorial Africa its incidence approaches 160 per 10,000, and in Malayan natives its incidence is over 15% (1500-2000 per 10,000).

Sex

• Being an almost wholly autosomal dominant disorder, there is no predilection towards either sex in hereditary elliptocytosis.
• The most important exception to this rule of autosomal dominant inheritance is for a subtype of hereditary elliptocytosis called hereditary pyropoikilocytosis (HPP). This condition is autosomal recessive.