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Sickle cell anaemia is the product of long term evolutionary pressures due to endemic malaria. The malaria parasite cannot survive in sickle cells, which is why the disease is so common among people who share African genes (where malaria is prevalent). The disease is inherited, but partially recessive, giving people that are heterozygous for the trait a huge advantage, as complications are less likely (there are many 'normal' red blood cells to make up for the anaemia that results from sickled cells), but they still have the protection against malaria.

The cause is a missense point mutation in the beta hemoglobin gene, which causes hemoglobin in red blood cells to aggregate under low oxygen conditions. This aggregation leads to the red blood cell becoming rigid and changing in shape to the characterisitic sickle shape that the disease takes its name from.

Although the disease succesfully protects against malaria, which has kept it endemic despite its high mortality rate, the rigid sickle shape cells can get caught up in blood vessels much more easily than normal cells, vastly increasing the risk and frequency of blood clots. This is a particular problem for people who are homozygous for sickle cell anaemia, since all of their red blood cells have this problem. Destruction of sickle cells by the body takes place in the pancreas, since the strangely shaped cells are not recognised as being 'real' red blood cells, which causes the anaemia.

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16y ago

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