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Sickle Cell Disorders

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Topic Overview

Some people inherit one sickle cell gene and one other defective hemoglobin gene, resulting in various types of sickling disorders. These disorders range from mild to severe.

  • Sickle cell disease (hemoglobin SS disease) occurs when both genes produce hemoglobin S. This person typically has symptoms of anemia, mild to life-threatening complications, and a shortened lifespan.
  • Sickle beta-thalassemia occurs when a person has one hemoglobin S gene and another gene that causes the body to produce less hemoglobin than normal. This person may have mild to severe sickle cell disease.
  • Hemoglobin SC disease occurs when a person has one hemoglobin S gene and one abnormal hemoglobin C gene. This person may have generally milder symptoms and a longer lifespan than a person with sickle cell disease but still may become seriously ill.
  • Hemoglobin SE disease occurs when a person has one hemoglobin S gene and one abnormal hemoglobin E gene. This person may have mild anemia. Most people do not have symptoms.
  • Hemoglobin SO disease and hemoglobin SD disease occur when a person has one hemoglobin S gene and one abnormal hemoglobin O or hemoglobin D gene. This person may experience all sickle cell disease symptoms, ranging from mild to severe.

References

Other Works Consulted

  • Natarajan K, et al. (2010). Disorders of hemoglobin structure: Sickle cell anemia and related abnormalities. In K Kaushansky et al., eds., Williams Hematology, 8th ed., pp. 709–741. New York: McGraw-Hill.
  • Wang WC (2009). Sickle cell anemia and other sickling syndromes. In JP Greer et al., eds., Wintrobe's Clinical Hematology, 12th ed., pp. 1038–1082. Philadelphia: Lippincott Williams and Wilkins.

Credits

ByHealthwise Staff
Primary Medical Reviewer E. Gregory Thompson, MD - Internal Medicine
Donald Sproule, MDCM, CCFP - Family Medicine
Adam Husney, MD - Family Medicine
Martin J. Gabica, MD - Family Medicine
Specialist Medical Reviewer Martin H. Steinberg, MD - Hematology

Current as ofOctober 9, 2017

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