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Summary
Sickle cell disease (SCD) is a rare blood disorder that is inherited in an autosomal recessive manner. It is characterized by the presence of sickle, or crescent-shaped, red blood cells (erythrocytes) in the bloodstream. These crescent-shaped cells are stiff and sticky and interact with other cells and the blood clotting system to block blood flow in the very tiny blood vessels (capillaries) of the peripheral blood system (blood vessels outside of the heart). This prevents the normal flow of nutrition and oxygen (as red blood cells are responsible for carrying oxygen throughout the body).
Common symptoms associated with SCD include excruciating bone pain, chest pain, severe infections (primarily in children), low levels of circulating red blood cells (anemia) and yellowing of the skin (jaundice). The blocked blood flow can also cause severe organ damage including stroke. SCD has several recognized forms including sickle cell anemia, sickle cell hemoglobin C disease, and sickle cell / beta-thalassemia disease.
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