Indication

Sickle cell disease

Sickle cell disease is a group of inherited red blood cell disorders characterized by the production of abnormal hemoglobin, primarily hemoglobin S. Read more about Sickle cell disease

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About Sickle cell disease.

Sickle cell disease is a group of inherited red blood cell disorders characterized by the production of abnormal hemoglobin, primarily hemoglobin S.

Under deoxygenated conditions, this abnormal hemoglobin polymerizes into rigid fibers, causing erythrocytes to distort into a crescent or sickle shape, lose flexibility, and undergo premature intravascular and extravascular hemolysis.

The impaired rheology of these deformed cells leads to microvascular occlusion, systemic endothelial inflammation, and tissue ischemia throughout the circulatory system.

Hallmarks include chronic hemolytic anemia, recurrent acute vaso-occlusive pain crises, increased susceptibility to encapsulated bacterial infections, and progressive multiorgan damage, such as acute chest syndrome and stroke.

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