Sickle Cell Disorders
- 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 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.
|Primary Medical Reviewer||E. Gregory Thompson, MD - Internal Medicine|
|Specialist Medical Reviewer||Martin Steinberg, MD - Hematology|
|Last Revised||October 7, 2010|
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