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 life span.
  • 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 life span 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.
  • Steinberg MH (2016). Sickle cell disease and other hemoglobinopathies. In L Goldman, A Shafer, eds., Goldman-Cecil Medicine, 25th ed., vol. 2, pp. 1095-1104. Philadelphia: Saunders.
  • 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 ReviewerE. Gregory Thompson, MD - Internal Medicine

Adam Husney, MD - Family Medicine

Martin J. Gabica, MD - Family Medicine

Specialist Medical ReviewerMartin Steinberg, MD - Hematology

Current as ofMarch 9, 2017

Current as of: March 9, 2017

Author: Healthwise Staff

Medical Review: E. Gregory Thompson, MD - Internal Medicine & Adam Husney, MD - Family Medicine & Martin J. Gabica, MD - Family Medicine & Martin Steinberg, MD - Hematology