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(Hereditary Hemochromatosis [HH]; Primary Hemachromatosis; Familial Hemochromatosis)
by Rosalyn Carson-DeWitt, MD
Hemochromatosis is a condition in which the body builds up too much iron. Two types of hemochromatosis are:
Primary or Hereditary Hemochromatosis (HH)
This type is caused by inherited genes and includes juvenile hemochromatosis. These genes cause the stomach and intestines to absorb too much iron. It is the most common genetic disorder in the US.
This type results from treatments or diseases that cause iron to build up in the body. This may include:
If found, early HH is easily treated. Untreated HH can lead to severe organ damage. Excess iron builds up in the cells of the liver, heart, pancreas, joints, and pituitary gland. This leads to diseases such as cirrhosis of the liver, liver cancer, diabetes, coronary artery disease, and joint disease.
HH is caused by a genetic defect. It is passed down through autosomal recessive inheritance. This means a child who inherits 2 genes, 1 from each parent, is very likely to develop HH. However, not all people who have 2 copies of the gene develop signs and symptoms of HH.
Risk Factors TOP
Hemochromatosis is usually present in men between 30-50 years old and in women over 50 years old (postmenopausal). Factors that may increase the chances of HH:
Many people have no symptoms when they are diagnosed. However when they occur, symptoms may include:
Complications may include:
If the disease is not treated early, iron may build up in body tissues. This may eventually lead to serious problems such as:
You will be asked about your symptoms and medical history. A physical exam will be done.
Tests may include:
Treatment is simple, inexpensive, and safe.
The first step is to rid the body of excess iron by removing blood. The schedule will depend on how severe the iron overload is. A pint of blood will be taken once or twice a week for several months to a year (or possibly longer). When iron levels return to normal, maintenance therapy is given. A pint of blood is given every 2-4 months for life. Some people may need it more often. Females may need to increase their schedule after menopause.
Lifestyle changes include steps to reduce the amount of iron you consume and/or absorb, and to help protect your liver:
Treating Associated Medical Conditions TOP
You may need to be treated for other conditions that have developed. Hemochromatosis can cause:
To help reduce the chances of hemochromatosis:
A genetic counselor can help you review your family history, determine your specific risks, and review the appropriate testing.
American Hemochromatosis Society
American Society of Hematology
Hemochromatosis. American Liver Foundation website. Available at: https://www.liverfoundation.org/for-patients/about-the-liver/diseases-of-the-liver/hemochromatosis. Accessed March 26, 2018.
Hemochromatosis. National Institute of Diabetes and Digestive and Kidney Diseases website. Available at:
...(Click grey area to select URL)
Updated March 2014. Accessed March 26, 2018.
Hemochromatosis. EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T116469/Hemochromatosis. Updated May 31, 2017. Accessed March 26, 2018.
Last reviewed March 2018 by EBSCO Medical Review Board Marcin Chwistek, MD
Last Updated: 5/1/2014
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