Hemochromatosis is a disease that causes excess iron in the body, which can accumulate in several organs of the body, and the appearance of complications such as cirrhosis of the liver, diabetes, darkening of the skin, heart failure, joint pain or dysfunction of the sexual glands, for example.
This disease can be caused in 2 ways:
- Hereditary Hemochromatosis: is the main cause of the disease, which happens due to mutations in the genes responsible for the absorption of iron in the digestive tract, which are now absorbed in large quantities; Secondary or Acquired Hemochromatosis: iron accumulation due to other situations, mainly in people with diseases called hemoglobinopathies, in which the destruction of red blood cells releases large amounts of iron into the bloodstream. Other causes are repeated blood transfusions, chronic cirrhosis or improper use of medications for anemia, for example.
The treatment for hemochromatosis is indicated by the hematologist, with phlebotomies, which are periodically drawn from blood so that the deposited iron is transferred to the new red blood cells that the body produces. Another option is the use of iron chelating remedies, such as Desferroxamine, which help its elimination.
Signals and symptons
The excess of iron in the bloodstream causes its deposit in several organs of the body such as liver, heart, pancreas, skin, joints, testicles, ovaries, thyroid and pituitary.
Thus, the main signs and symptoms that may arise include:
- Fatigue; Weakness; Cirrhosis of the liver; Diabetes; Heart failure and arrhythmias; Joint pain; Infertility; Absence of menstruation; Sexual impotence; Hypothyroidism.
In addition, the accumulation of iron and fibrosis of the liver increases the chances of developing liver cancer. Read more about the symptoms that indicate excess iron.
How to confirm
The main tests indicated for the diagnosis are:
- Dosages of iron, ferritin, blood transferrin saturation. Know what ferritin is and how to evaluate this test; Genetic tests, which can show changes in the genes that cause the disease; Liver biopsy, especially when it has not yet been possible to confirm the disease or to prove the deposit of iron in the liver; Response test to phlebotomy, done with the withdrawal of blood and monitoring of iron levels, indicated mainly for people who cannot undergo a liver biopsy or where there are still doubts about the diagnosis;
The hematologist will also be able to request measurements of liver enzymes, investigate the function or deposit of iron in organs that may be affected, as well as exclude other diseases that may cause similar symptoms.
Hemochromatosis should be investigated in people who have suggestive symptoms, when there is unexplained liver disease, diabetes, heart disease, sexual dysfunction or joint disease, and also in people who have first-degree relatives with the disease or who have changes in the rates of blood tests iron.
How the treatment is done
Hereditary hemochromatosis has no cure, however, treatment can be done as a way to decrease iron stores in the blood and prevent deposits in the organs.
The main form of treatment is with phlebotomies, also called bleeds, in which part of the blood is removed in sessions so that the excess iron becomes part of the new red blood cells that the body produces.
This treatment has a more aggressive initial session, but maintenance doses are required, in which approximately 350 to 450 ml of blood are taken 1 to 2 times a week. Then, the sessions can be spaced according to the result of the follow-up exams, indicated by the hematologist.
Another treatment option is the use of iron chelators or "scavengers", such as Desferroxamine. This treatment is indicated for people who cannot tolerate phlebotomy, especially those with severe anemia, heart failure or advanced liver cirrhosis. Learn more treatment guidelines for excess iron in your blood.
Hemochromatosis Diet
Throughout the treatment, it is also indicated to reduce the consumption of excess iron through food. Some diet tips are:
- Avoid eating meat in large quantities, giving preference to white meat; Eat fish at least twice a week; Avoid eating iron-rich vegetables, such as spinach, beets or green beans, more than once a week; Eat whole grain bread instead of white or iron-enriched bread; eat cheese, milk or yogurt daily because calcium slows down iron absorption; avoid eating nuts, such as raisins, in large quantities because it is rich in iron.
In addition, the patient should avoid alcoholic beverages in order to avoid liver damage and not to consume vitamin supplements with iron and vitamin C, as this increases the absorption of iron. Find out more about the foods you should avoid at Foods rich in iron.
