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SICKLE CELL DISEASE AND OTHER HEMOGLOBINOPATHIES

Source: www.idph.state.ia.us
Topic: Sickle Cell Disease
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Sort Desciption: Definition Sickle cell diseases comprise a group of genetic disorders characterized by the presence of hemoglobin S (Hb S). Hb S can cause red blood cells to change from their usual biconcave disc shape to a crescent or sickle shape during deoxygenation. ...

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Definition Sickle cell diseases comprise a group of genetic disorders characterized by the presence of hemoglobin S (Hb S). Hb S can cause red blood cells to change from their usual biconcave disc shape to a crescent or sickle shape during deoxygenation. The red blood cell resumes a normal configuration, but after repeated cycles of sickling and unsickling, the red blood cell becomes damaged permanently, and hemolysis occurs. This hemolysis is responsible for the anemia that is the hallmark of sickle cell disease. Incidence Sickle cell disease affects 1 in 375 African-Americans and more than 1 in 50,000 Americans. It is estimated that 8% of the African American population carries the sickle cell trait. The disease can also affect those of Mediterranean, Caribbean, South and Central American, Arabian, or East Indian ancestry. Inheritance Sickle hemoglobin (Hb S) is inherited as autosomal recessive from both parents or Hb S from one parent and a gene for an abnormal hemoglobin or beta-thalassemia from the other parent. Characteristics Acute and chronic tissue injury can occur when blood flow through the vessels is obstructed by abnormally shaped red cells. Complications include painful episodes involving soft tissues and bones, acute chest syndrome, priapism, cerebral vascular accidents, and both splenic and renal dysfunction. Common causes of mortality among children with sickle cell disease include bacterial infections, splenic sequestration crisis, and acute chest syndrome. Variant Forms Types of sickle cell disease include sickle cell anemia (SS), hemoglobin SC disease, sickle beta thalassemia, and SE disease. Other hemoglobin diseases include hemoglobin CC and EE diseases. Newborn Screening Methodology The laboratory method used is Isoelectric Focusing (IEF) and High Performance Liquid Chromatography (HPLC). Confirmation Health care providers should seek consultatio ...

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