What is hemochromatosis?
Hemochromatosis is an inherited blood disorder that causes the body to retain excessive amounts of iron. This iron overload can lead to serious health consequences, most notably cirrhosis of the liver. Hemochromatosis is hereditary. Men and women are equally affected by hemochromatosis, but women are diagnosed later in life because of blood loss from
menstruation and childbirth. It most commonly appears in patients between the ages of 40–60, since it takes many years for the body to accumulate excessive iron.
Hemochromatosis is also known as iron overload, bronze diabetes, hereditary hemochromatosis and familial hemochromatosis. The inherited disorder causes increased absorption of intestinal iron, well beyond that needed to replace the body's loss of iron. Iron helps make and maintain red blood cells, the cells that transport oxygen throughout the body. The body must regulate the amount of iron in its system because too much iron or too little iron can be problematic. The body maintains a balance of iron by absorbing small amounts of iron necessary to replenish equal amounts lost. Patients with hemochromatosis absorb more iron than the body can eliminate. As a result, the body stores high levels of iron that begin to accumulate in the cells. Eventually, the excess iron causes tissue and organ damage
Hemochromatosis causes excess iron storage in several organs of the body including the liver, pancreas, endocrine glands, heart, skin, and intestinal lining. The buildup of iron in these organs can lead to serious complications, including heart failure, liver cancer, andcirrhosis of the liver. It is estimated that about 5% of cirrhosis cases are caused by hereditary hemochromatosis.
Idiopathic pulmonary hemosiderosis, a disorder afflicting children and young adults, is a similar overload disorder characterized by abnormal accumulation of hemosiderin. Hemosiderin is a protein found in most tissues, especially the liver. It is produced by digestion of hematin, an iron-related substance.
More information on hemochromatosis
What is hemochromatosis? - Hemochromatosis is an inherited blood disorder also known as iron overload, bronze diabetes, hereditary hemochromatosis and familial hemochromatosis.
What causes hemochromatosis? - Hemochromatosis is caused by a defect in a gene called HFE, which helps regulate the amount of iron absorbed from food.
What are the risk factors for hemochromatosis? - The risk factors for hemochromatosis are alcohol, diet, gender, blood loss, and mutation type.
What are the symptoms of hemochromatosis? - The symptoms of hemochromatosis include fatigue, weight loss, weakness, shortness of breath, heart palpitations, chronic abdominal pain, and impaired sexual performance.
How is hemochromatosis diagnosed? - The most common diagnostic methods for hemochromatosis are blood tests and liver biopsy. A liver biopsy often is recommended to confirm the diagnosis.
What are the treatments for hemochromatosis? - The most effective treatment of hemochromatosis is to reduce the blood iron by removing blood using phlebotomy (withdrawal of blood from the arm veins).