<Tr> <Th> MeSH </Th> <Td> D013103 </Td> </Tr> <Tr> <Td_colspan="2"> (edit on Wikidata) </Td> </Tr> <P> Hereditary spherocytosis (also known as Minkowski--Chauffard syndrome) abnormality of erythrocytes . The disorder is caused by mutations in genes relating to membrane proteins that allow for the erythrocytes to change shape . The abnormal erythrocytes are sphere - shaped (spherocytosis) rather than the normal biconcave disk shaped . Dysfunctional membrane proteins interfere with the cell's ability to be flexible to travel from the arteries to the smaller capillaries . This difference in shape also makes the red blood cells more prone to rupture . Cells with these dysfunctional proteins are taken for degradation at the spleen . This shortage of erythrocytes results in hemolytic anemia . </P> <P> It was first described in 1871 and is the most common cause of inherited hemolysis in Europe and North America within the Caucasian population, with an incidence of 1 in 5000 births . The clinical severity of HS varies from symptom - free carrier to severe haemolysis because the disorder exhibits incomplete penetrance in its expression . </P>

Hereditary spherocytosis occurs due to mutation in genes coding for