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.
- 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.
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 Steinberg, MD - Hematology
Current as ofOctober 13, 2016
