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 Reviewer E. Gregory Thompson, MD – Internal Medicine
Adam Husney, MD – Family Medicine
Martin J. Gabica, MD – Family Medicine
Specialist Medical Reviewer Martin Steinberg, MD – Hematology

Current as ofOctober 9, 2017