Hereditary spherocytosis epidemiology and demographics

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

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Hereditary spherocytosis can present at any age with any presentation from hydrops fetalis inutero through diagnosis in the ninth decade of life, and is reported worldwide in all racial and ethnic groups. It is most common inherited anemia in northern european ancestry and north america. The reported incidence is 1 in 2000 births. Approximately 25% of all hereditary spherocytosis is autosomal recessive. It is most often diagnosed in childhood or early adulthood.

• Hereditary spherocytosis is reported worldwide in all racial and ethnic groups.^[1]
• It is the most common inherited anemia in the northern European ancestry and north america.^[2]
• The reported incidence of hereditary spherocytosis is 1 in 2000 births.^[3]
• It is less commonly seen in african american and southeast asian people.^[4]

• In the United States, the incidence of the disorder is approximately one case in 5000 people.
• Given that approximately 25% of all hereditary spherocytosis is autosomal recessive, calculations indicate that 1.4% of the US population might be silent carriers of hereditary spherocytosis.

• In northern European, hereditary spherocytosis affects as many as 1 in 2000 to 1 in 5000 (prevalence, approximately 0.02 to 0.05 percent) [6,7,62,75].
• The frequency is thought to be lower in individuals from other parts of the world such as Africa and Southeast Asia, although comprehensive population survey data are unavailable.

• Hereditary spherocytosis can present at any age and with any severity, with case reports describing a range of presentations, from hydrops fetalis in utero through diagnosis in the ninth decade of life.^[5][6]
• Hereditary spherocytosis is most often diagnosed in childhood or early adulthood.
• Children diagnosed early in life usually have a severe form of hereditary spherocytosis that results in their early presentation. Jaundice is likely to be most prominent in newborns. The magnitude of hyperbilirubinemia may be such that exchange transfusion is required. Approximately 30-50% of adults with hereditary spherocytosis had a history of jaundice during the first week of life. Recognition of hereditary spherocytosis as a potential cause of neonatal anemia and hyperbilirubinemia and institution of prompt treatment may reduce the risk of bilirubin-induced neurologic dysfunction in these patients.^[7]

• Hereditary spherocytosis occurs in all racial and ethnic groups but is more common in northern Europeans,

• There is no significant data from US related to gender difference. In 2011, the number of cases was 114 reported from CBM(China Biology Medicine) database, the male: female ratio was 1.04:1.^[8]
• In 2011, overall literature reported prevalence of hereditary spherocytosis in China was estimated to be: 1.27 cases per 100,000 people in males and 1.49 cases per 100,000 people in females

• Hereditary spherocytosis occurs in 1 in 5,000 individuals of Northern European ancestry. This condition is the most common cause of inherited anemia in that population. The prevalence of hereditary spherocytosis in people of other ethnic backgrounds is unknown, but it is much less common.

There is no particular relation of FA with developed countries.

There is no particular relation of FA with developing countries.

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