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Autosplenectomy can occur in cases of sickle-cell disease where the misshapen cells block blood flow to the spleen, causing scarring and eventual atrophy of the organ. [2] Autosplenectomy is a rare condition that is linked to certain diseases but is not a common occurrence. It is also seen in systemic lupus erythematosus (SLE).
In Sudan, sickle cell disease was first reported in 1926 by Archibald. [23] The disease is considered one of the major types of anemia, especially in Western Sudan where the sickle cell gene is frequent [24] Sickle cell disease is the major haemoglobinopathy seen in Khartoum, the capital of Sudan. This may be attributed to the migration of ...
This would be similar to the hemoglobin allele which is responsible for sickle-cell disease, but solely in people with two copies; those with just one copy of the allele have a sickle cell trait and gain partial immunity to malaria as a result. This effect is called heterozygote advantage. [27]
A sickle allele is always the same mutation of the beta-globin gene (glutamic acid to valine at amino acid six). In contrast, beta-thalassemia alleles can be created by many different mutations including both deletion and non-deletion forms.
The correlation between sickle-cell disease and malaria is a double-edged sword. Having a sickle-cell allele does limit the life expectancy of a person, however, the presence of sickle-cell genes reduces the detrimental effects of malaria should it be contracted.
Diamond–Blackfan anemia (DBA) is a congenital erythroid aplasia that usually presents in infancy. [3] DBA causes low red blood cell counts (), without substantially affecting the other blood components (the platelets and the white blood cells), which are usually normal.