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Alternative Names Return to topG-6-PD deficiency; Hemolytic anemia due to G6PD deficiency; Anemia - hemolytic due to G6PD deficiency
Definition Return to top
Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency is a hereditary condition in which red blood cells break down when the body is exposed to certain drugs or the stress of infection.
Causes Return to top
G6PD deficiency occurs when a person is missing or doesn't have enough of an enzyme called glucose-6-phosphate dehydrogenase, which helps red blood cells work properly.
Too little G6PD leads to the destruction of red blood cells. This process is called hemolysis. When this process is actively occurring, it is called a hemolytic episode. The episodes are usually brief, because the body continues to produce new red blood cells, which have normal activity.
Red blood cell destruction can be triggered by infections, severe stress, certain foods (such as fava beans), and certain drugs, including:
Other chemicals, such as those in mothballs, can also trigger an episode.
In the United States, G6PD deficiency is more common among blacks than whites. Men are more likely to have this disorder than women.
You are more likely to develop this condition if you:
A form of this disorder is common in whites of Mediterranean descent. This form is also associated with acute episodes of hemolysis. Episodes are longer and more severe than in the other types of the disorder.
Symptoms Return to top
Persons with this condition do not display any signs of the disease until their red blood cells are exposed to certain chemicals in food or medicine, or to stress.
Symptoms are more common in men and may include:
Exams and Tests Return to top
A blood test can be done to check the level of G6PD. See: G6PD screen
Other tests that may be done include:
Treatment Return to top
Treatment may involve:
Outlook (Prognosis) Return to top
Spontaneous recovery from hemolytic crises is the usual outcome.
Possible Complications Return to top
Rarely, kidney failure or death may occur following a severe hemolytic event.
When to Contact a Medical Professional Return to top
Call for an appointment with your health care provider if you have symptoms of this condition.
Call your health care provider if you have been diagnosed with G6PD deficiency and symptoms do not disappear after treatment.
Prevention Return to top
Persons with G6PD deficiency must strictly avoid things that can trigger an episode. Talk to your health care provider about your medications.
Genetic counseling or testing may be available to those who have a family history of the condition.
References Return to top
Hoffman R, Benz Jr. EJ, Shattil SJ, et al., eds. Hematology: Basic Principles and Practice. 4th ed. Philadelphia, Pa: Churchill Livingston; 2005:658-60.
Goldman L, Ausiello D. Cecil Textbook of Medicine. 22nd ed. Philadelphia, Pa: WB Saunders; 2004:1027-28.Update Date: 11/10/2008 Updated by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and James R. Mason, MD, Oncologist, Director, Blood and Marrow Transplantation Program and Stem Cell Processing Lab, Scripps Clinic, Torrey Pines, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.