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Sickle cell-beta thalassemia is an inherited blood disorder. The disease may range in severity from being relatively benign and like sickle cell trait to being similar to sickle cell disease . [ 1 ] [ 2 ]
The person may need to be monitored for several days in the hospital after the transfusion, but the length of stay generally depends on the condition for which the exchange transfusion was performed. Sickle Cell Disease patients may be exchanged in an outpatient setting and can be sent home the very same day. [13]
Sickle cell disease, in which a mutation in the globin gene causes the formation of sickle hemoglobin. [2] This disease is marked by the manifestation of chronic compensated hemolytic anemia , with laboratory findings not limited to unconjugated hyperbilirubinemia but also elevated serum lactate dehydrogenase and low serum haptoglobin .
The association with kidney and lung infarcts was noted in 1931 by Yater and Mollari and Baird in 1934 respectively. The term sickle-cell trait was coined by Samuel Diggs in Memphis in 1933 to distinguish heterozygotes from those with sickle-cell anaemia. Diggs also reported the association with splenic fibrosis in 1935.
Due to underlying diseases that destroy the spleen (autosplenectomy), e.g. sickle-cell disease. Celiac disease: unknown physiopathology. [6] In a 1970 study, [7] it was the second most common cause of abnormalities of red blood cells linked to hyposplenism, after surgical splenectomy.
Dactylitis can occur in seronegative arthropathies, such as psoriatic arthritis and ankylosing spondylitis, and in sickle-cell disease as result of a vasoocclusive crisis with bone infarcts, and in infectious conditions including tuberculosis, syphilis, and leprosy. In reactive arthritis, sausage fingers occur due to synovitis. [2]
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