Sickle Cell Disorders
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.
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 (2012). Sickle cell disease and other hemoglobinopathies. In L Goldman, A Shafer, eds., Goldman's Cecil Medicine, 24th ed., pp. 1066–1075. 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.
|Primary Medical Reviewer||E. Gregory Thompson, MD - Internal Medicine|
|Specialist Medical Reviewer||Martin Steinberg, MD - Hematology|
|Last Revised||October 1, 2012|