Materiały źródłowe

• #1

  https://www.nhs.uk/conditions/haemochromatosis/treatment/

  There’s currently no cure for haemochromatosis, but there are treatments that can reduce the amount of iron in your body. […] The most commonly used treatment for haemochromatosis is a procedure to remove some of your blood, known as a venesection or phlebotomy. […] There are 2 main stages to treatment: induction blood is removed on a frequent basis (usually weekly) until your iron levels are normal; this can sometimes take up to a year or more […] maintenance blood is removed less often (usually 2 to 4 times a year) to keep your iron levels under control; this is usually needed for the rest of your life. […] A treatment called chelation therapy may be used in a small number of cases where regular phlebotomies are not possible because it’s difficult to remove blood regularly for example, if you have very thin or fragile veins. […] Commonly used medicines are deferasirox and desferrioxamine. […] Deferasirox is unlicensed for the treatment of haemochromatosis, which means it has not undergone extensive clinical trials for this use. But your doctor may recommend it if they feel the possible benefits outweigh any risks.

• #1 Treatment of Hemochromatosis – NIDDK

  https://www.niddk.nih.gov/health-information/liver-disease/hemochromatosis/treatment

  In most cases, doctors treat hemochromatosis with phlebotomy, or drawing about a pint of blood at a time, on a regular schedule. This is the most direct and safe way to lower body stores of iron. […] Treatment of hemochromatosis can improve symptoms and prevent complications. […] Phlebotomy removes extra iron from your blood. Phlebotomy is simple, inexpensive, and safe. […] After phlebotomy has removed extra iron and blood levels of iron and ferritin return to normal, doctors will reduce phlebotomies to once every 1 to 3 months and eventually to 2 to 3 times a year. […] To treat secondary hemochromatosis in these people, doctors prescribe medicines, called chelating agents, that bind to iron and allow it to pass from the body in urine. […] Doctors treat neonatal hemochromatosis in newborns with exchange transfusions removing blood and replacing it with donor blood and IV immunoglobulin a solution of antibodies from healthy people.

• #1 Hemochromatosis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hemochromatosis/diagnosis-treatment/drc-20351448

  Medical professionals can treat hemochromatosis safely and effectively by removing blood from the body on a regular basis. This is similar to donating blood. The process is known as phlebotomy. […] The goal of phlebotomy is to lower the iron levels. The amount of blood removed and how often it’s removed depend on age, overall health and the severity of iron overload. […] Treating hemochromatosis can help relieve symptoms of tiredness, stomach pain and skin darkening. It can help prevent serious complications such as liver disease, heart disease and diabetes. If you already have one of these conditions, phlebotomy may slow the progression of the disease. In some cases, it may even reverse it. […] Phlebotomy can’t reverse cirrhosis or joint pain, but it can slow the progression. […] Phlebotomy may not be an option for someone who has certain conditions, such as anemia or heart complications. Instead, a healthcare professional may recommend a medicine to remove excess iron. The medicine can be injected into the body, or it can be taken as a pill. The medicine binds excess iron, allowing the body to expel iron through urine or stool in a process that’s called chelation (KEE-lay-shun). Chelation is not commonly used in hemochromatosis.

• #1 Patient education: Hereditary hemochromatosis (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/hereditary-hemochromatosis-beyond-the-basics/print

  The decision to begin phlebotomy in a person with hemochromatosis is usually based on a person’s age, sex, level of ferritin in the blood, and results of magnetic resonance imaging (MRI) or liver biopsy. When iron overload is present, phlebotomy should be started. […] Phlebotomy is appropriate and beneficial for most people with excess iron stores from hemochromatosis, including older adults and people who have no symptoms. […] Blood counts and ferritin levels are usually monitored every 4 weeks during treatment. These values help to determine when the excess iron stores have been depleted; they also help determine if phlebotomy has caused anemia by depleting iron too rapidly. […] In people with hemochromatosis who do not have symptoms at the time of diagnosis, the excess iron stores are removed after approximately 15 to 30 or fewer phlebotomy treatments. In people who have symptoms at the time of diagnosis, 50 or more phlebotomy treatments may be needed to deplete excess iron stores.

• #1 Sign up for our monthly newsletter

  https://www.haemochromatosis.org.uk/genetic-haemochromatosis-care-pathway

  Following a period of regular venesection, you will reach maintenance once your serum ferritin (SF) is below 100 g/l and transferrin saturation (TSAT) is below 50%. […] You will need to continue to have venesection periodically to maintain your iron levels at normal levels, ideally where serum ferritin (SF) is below 50 g/l and transferrin saturation (TSAT) is below 50%. […] Once in maintenance, many people can go on to be regular blood donors, helping others.

• #1 Hemochromatosis Treatment & Management: Approach Considerations, Surgical Intervention, Phlebotomy

  https://emedicine.medscape.com/article/177216-treatment

  Deferasirox (Exjade) is the oral iron chelator that should be taken once daily as an adjunct to phlebotomies or instead of phlebotomy in patients in whom these procedures are poorly tolerated. […] The first patient affected by juvenile hemochromatosis was successfully treated with chelation therapy. […] Patients affected with anemia cannot be treated with phlebotomy. Thus, application of iron chelation agents (eg, deferoxamine, deferiprone, deferasirox) is recommended. […] Dietary changes intended to minimize or eliminate iron ingestion are usually unnecessary and are often not feasible. […] Regular monitoring of hematocrit, hemoglobin, and serum ferritin levels is necessary in patients undergoing phlebotomy.

• #1 What is haemochromatosis? – BHF

  https://www.bhf.org.uk/informationsupport/heart-matters-magazine/medical/haemochromatosis

  Haemochromatosis is an inherited condition which results in too much iron being stored in the body. […] Most commonly we remove the iron with repeated venesection, which means removing blood. This is very like what happens when you donate blood. Your body will make up the blood that is lost using some of your iron stores. So having repeated venesections will result in a gradual reduction of your iron stores. […] Once the ferritin is low then you move onto the maintenance programme. Here you will often need venesection every 3-4 months, or sometimes it may be longer gaps. […] You can donate blood through the blood service if you are eligible to do so once you reach the maintenance phase. […] Sometimes due to heart problems or co-existing blood problems, venesection may be unsuitable. In this case medication, called chelation, can be used to make the iron come out in the urine or stool. These are not used often, as they are expensive, have side effects and need close monitoring.

• #1 Iron overload – Wikipedia

  https://en.wikipedia.org/wiki/Iron_overload

  Phlebotomy is associated with improved survival if it is initiated before the onset of cirrhosis or diabetes. […] The human diet contains iron in two forms: heme iron and non-heme iron. Heme iron is usually found in red meat, whereas non-heme iron is found in plant based sources. Heme iron is the most easily absorbed form of iron. In those with hemochromatosis undergoing phlebotomy for treatment; restriction of dietary iron is not required. However, those who do restrict dietary iron usually require less phlebotomy (about 0.5-1.5 liters of blood less per year). Vitamin C and iron supplementation should be avoided as vitamin C accelerates intestinal absorption of iron and mobilization of body iron stores. Alcohol consumption should be avoided due to the risk of compounded liver damage with iron overload.

• #1

  https://www.nhs.uk/conditions/haemochromatosis/

  There’s currently no cure for haemochromatosis, but there are treatments that can reduce the amount of iron in the body and reduce the risk of damage. […] There are 2 main treatments. […] venesection (phlebotomy) a procedure to remove some of your blood; this may need to be done every week at first and can continue to be needed 2 to 4 times a year for the rest of your life […] chelation therapy where you take medicine to reduce the amount of iron in your body; this is only used if it’s not easy to regularly remove some of your blood. […] You do not need to make any big changes to your diet to control your iron levels if you’re having treatment, but you’ll usually be advised to avoid: breakfast cereals containing added iron, iron or vitamin C supplements, drinking too much alcohol.

• #1 Patient education: Hereditary hemochromatosis (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/hereditary-hemochromatosis-beyond-the-basics/print

  Dietary considerations — People who are receiving treatment for hemochromatosis do not have to follow a special diet. There is no evidence that the condition is worsened by consuming moderate amounts of iron-rich foods such as red meat and liver. […] Chelation therapy refers to treatment with a drug that removes iron from the body. Examples are deferoxamine, deferasirox, or deferiprone. These drugs tightly bind iron and remove it from the body, lowering iron stores. […] Although phlebotomy can alleviate or even completely resolve some complications of hemochromatosis, other measures may be necessary to treat complications that persist.

• #1 Treatment and Maintenance – Canadian Hemochromatosis Society

  https://www.toomuchiron.ca/hemochromatosis/treatment-and-maintenance/

  In the active de-ironing phase, reducing iron intake is almost insignificant compared to the 225 mg of iron that are being removed with each phlebotomy. In the maintenance phase, restricting iron intake may increase the time between phlebotomies but at no time will reducing iron in the diet replace phlebotomies as effective therapy.

• #1 Pathogenesis, Diagnostics, and Treatment of Hereditary Haemochromatosis: A 150 Year-Long Understanding of an Iron Overload Disorder – European Medical Journal

  https://www.emjreviews.com/hepatology/article/pathogenesis-diagnostics-and-treatment-of-hereditary-haemochromatosis-a-150-year-long-understanding-of-an-iron-overload-disorder/

  Currently, the treatment of HH is based on phlebotomy therapy (venesection) and the elimination of iron by chelation therapy, depending on the aetiology. To date, phlebotomy is preferred, as it is more effective and more pleasant for the patient. […] The treatment should be done on a regular weekly basis, usually for 2 years. After reaching the desired iron decrease that is indicated by levels of serum ferritin, the patient undergoes (for the rest of their lives) maintenance phlebotomy every few months that is controlled by monitoring of transferrin saturation (ideally 50%) and serum ferritin (desired level 50-100 g/L). […] Iron chelation is also a method to reduce iron pools in patients, particularly in those in which severe physical or medical difficulties in giving blood prevail. […] More recent findings suggest that the treatment with erythrocytapheresis is an alternative treatment. […] Hepcidin-targeted therapies in which novel therapeutics manipulate the mechanisms regulating hepcidin production could provide a more fundamental future approach in HH treatment. […] During any of the above-mentioned treatments, the reduction of iron overload is also managed by dietary changes.

• #1 Recent advances in the treatment of iron overload – Hospital Pharmacy EuropeHospital Pharmacy Europe

  https://hospitalpharmacyeurope.com/news/editors-pick/recent-advances-in-the-treatment-of-iron-overload/

  Genetic haemochromatosis Venesections (ie, repeated phlebotomies) remain the reference method for treating iron excess in these diseases. […] Therefore, therapeutic research is now based upon designing forms of hepcidin that could be administered to patients in order to counteract hepcidin deficiency, which causes iron excess. Such a therapeutic approach would be ideal to avoid both iron excess reconstitution (after iron depletion has been obtained by initial venesections) or to prevent the development of body iron excess in case of early diagnosis. […] Another therapeutic way would be to find drugs counteracting digestive iron hyperabsorption and/or excessive iron release out of the macrophages by inhibiting membrane iron transporters. […] Whether deferasirox could be an adjunct or even replace venesections in genetic haemochromatosis needs to be evaluated.

• #1 Hemochromatosis Treatment & Management: Approach Considerations, Surgical Intervention, Phlebotomy

  https://emedicine.medscape.com/article/177216-treatment

  The AASLD guidelines state hereditary hemochromatosis patients who have evidence of iron overload are strongly encouraged to receive phlebotomy regularly until iron stores are depleted. […] In a retrospective analysis of 12 paired patients from the Netherlands with hereditary hemochromatosis homozygous for the C282Y mutation, those who received proton pump inhibitors (PPIs) had a significant reduction in the frequency of median number of phlebotomies compared with before the administration of PPIs. […] One study showed that phlebotomy therapy can reduce liver fibrosis, and the effects of therapy are dependent on the stage of the disease. […] Phlebotomy is generally a safe and efficient method of iron removal. […] In patients with hemochromatosis and heart disease, anemia, or poor venous access, treatment with iron chelation agents is recommended.

• #1 Patient education: Hereditary hemochromatosis (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/hereditary-hemochromatosis-beyond-the-basics

  In people with hemochromatosis who do not have symptoms at the time of diagnosis, the excess iron stores are removed after approximately 15 to 30 or fewer phlebotomy treatments. […] Maintenance phlebotomy — After excess iron has been removed, maintenance phlebotomy is used to prevent iron from reaccumulating. […] Effectiveness of phlebotomy — Phlebotomy can effectively remove iron and resolve some, but not all, complications of hemochromatosis. […] Chelation therapy (deferoxamine or deferasirox treatment) — Chelation therapy refers to treatment with a drug that removes iron from the body. […] Treatment of complications — Although phlebotomy can alleviate or even completely resolve some complications of hemochromatosis, other measures may be necessary to treat complications that persist.

• #1 Hemochromatosis | Cleveland Clinic

  https://my.clevelandclinic.org/departments/digestive/medical-professionals/hepatology/hemochromatosis

  The treatment of HH is simple and relatively safe. Therapeutic phlebotomy will effectively mobilize and remove iron stores and, when adhered to on a regular basis, will maintain them at normal levels. Patients should be encouraged to adhere to a regimen of phlebotomy of one unit of blood once or twice weekly as tolerated initially. This will remove approximately 250 mg of iron for each unit of phlebotomy, depending on the starting hematocrit value. In situations in which total body iron stores exceed 20 to 30 g, this regimen of phlebotomy may take up to 2 to 3 years to complete. The aim is to reduce iron stores to a level just short of iron deficiency. The hematocrit value should be monitored before each phlebotomy and should be postponed if it falls by more than 20% of its starting value. It is reasonable to check the serum ferritin level after every 10 to 12 phlebotomies. The serum ferritin level may be expected to fall progressively with iron mobilization, and it can be confidently assumed that effective mobilization of the iron stores will be completed when the serum ferritin level falls below 50 ng/mL.

• #1 Haemochromatosis – British Liver Trust

  https://britishlivertrust.org.uk/information-and-support/liver-conditions/haemochromatosis/

  An enlarged liver may reduce in size but if cirrhosis has become advanced, improvement is unlikely. If you have cirrhosis, doctors may run blood tests and imaging tests at regular intervals (usually every six months). Having cirrhosis will put you at a much higher risk of developing hepatocellular carcinoma (HCC). If this occurs, a liver transplant may be required. […] In diabetes, phlebotomy will not be able to repair damage to your pancreas. Other serious problems such as arthritis and sexual disorders arising from damage to the pituitary gland are unfortunately unlikely to improve. Symptoms such as tiredness and abdominal pain should lessen with recovery. The colour of your skin should return to normal and if you have heart disease, such as cardiomyopathy, any improvement will be linked to the severity of any damage caused by haemochromatosis.

• #1 Hereditary Hemochromatosis – Hematology and Oncology – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/hematology-and-oncology/iron-overload/hereditary-hemochromatosis

  Treatment is usually with serial phlebotomies. […] Treatment is indicated for patients with clinical manifestations, elevated serum ferritin levels (particularly levels 1000 ng/mL [ 1000 mcg/L]), or elevated transferrin saturation 45%. […] Phlebotomy is the simplest and most effective method to remove excess iron. It delays progression of fibrosis to cirrhosis, sometimes even reversing cirrhotic changes, and prolongs survival, but it does not prevent hepatocellular carcinoma. […] Approximately 500 mL of blood (approximately 250 mg of iron) is removed weekly or every other week until serum ferritin levels reach 50 to 100 ng/mL (50 to 100 mcg/L). […] When iron levels are normal, phlebotomy can be done intermittently to maintain the ferritin level between 50 and 100 ng/mL. […] Diabetes mellitus, cardiomyopathy, erectile dysfunction, and other secondary manifestations are treated as indicated.

• #1 Haemochromatosis

  https://dermnetnz.org/topics/haemochromatosis

  Treatment of haemochromatosis consists of regular removal of blood and is similar to making a blood donation (venesection therapy or phlebotomy). Every mL removed contains a 0.5 g of iron. Depending on the amount of iron overload the procedure may initially be performed once weekly or once monthly. This regularity of treatment continues until serum ferritin levels fall back to normal; this may take up to 2 years or more. After this, lifelong maintenance therapy needs to be set in place as excess iron continues to be absorbed. On average venesection is required every 34 months to prevent build-up and maintain healthy levels. Regular monitoring of serum ferritin, transferrin saturation, haematocrit and haemoglobin is necessary throughout the treatment process. […] It is essential to detect haemochromatosis as early as possible so that venesection can be instituted to prevent the build-up of iron and potential complications. Once complications such as diabetes and cirrhosis have developed, they cannot be reversed. Tiredness and abdominal pain should diminish, and increased pigmentation of the skin should fade over time once treatment has started. Arthritis, however, may fail to improve with venesection.

• #1 Hemochromatosis (Iron Overload): Causes, Symptoms, Treatment, Diet & More

  https://my.clevelandclinic.org/health/diseases/14971-hemochromatosis-iron-overload

  The outlook for hemochromatosis depends on the timing of diagnosis and treatment. If not caught and addressed early, severe hemochromatosis can cause serious problems. These complications can include organ damage and possible death. […] But hemochromatosis is also a manageable disease. With early detection and treatment, you can survive and live a normal, healthy life. Sometimes organ damage can even be reversed. […] Your healthcare provider will determine how long you need treatment. […] If you are diagnosed with hemochromatosis, you’ll need to have some blood withdrawn once a week to start. You may then be able to switch to every few months, though you’ll still need ongoing treatment. […] Hemochromatosis is a common disease that makes the body store too much iron. Although it can cause serious problems, its very treatable, especially when identified early. Talk to your healthcare provider if you have symptoms of hemochromatosis or a family history of it. Genetic testing can identify the disease early so you can live a long, healthy life.

• #1 Haemochromatosis | Better Health Channel

  https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/haemochromatosis

  Treatment includes regularly removing blood until iron levels normalise. […] A person with haemochromatosis is treated with venesection. This is a procedure similar to blood donation, where around 500 mls of blood is removed until iron in the blood is reduced to normal levels. Depending on the severity of the condition, this may take around one and a half years of twice-weekly visits. Once iron levels are normal, venesection needs to be performed three or four times every year for life. If haemochromatosis is treated in its earliest stages before severe organ damage has occurred, there is no reduction in life expectancy other things being equal.

• #1 Treatment and Maintenance – Canadian Hemochromatosis Society

  https://www.toomuchiron.ca/hemochromatosis/treatment-and-maintenance/

  In the initial phase of de-ironing, depending on the amount of stored iron, phlebotomies may need to occur once a week or even twice a week for one year. […] During the maintenance phase of treatment, the goal is to keep transferrin saturation between 30-40% while maintaining a normal hemoglobin (normal hemoglobin range is 140-180 g/L for men and 120-160 g/L for women). It depends on the individual, but typically this could be achieved with one phlebotomy every 3-4 months. The treatment is ongoing for life. […] Studies clearly show that both survival rates and quality of life are significantly improved with phlebotomy therapy, even for patients who have already sustained organ damage. […] In the future, patients with hemochromatosis may have other options for the type of treatment that is best for them, but for now, phlebotomy is the gold standard.

• #1 Haemochromatosis | Leukaemia & Blood Cancer New Zealand

  https://www.leukaemia.org.nz/information/about-blood-cancers/haemochromatosis/

  Not everyone with haemochromatosis will need treatment. However, once diagnosed, you are likely to need regular blood tests. If you have a high ferritin level, then you may need treatment to prevent organ damage. […] Treatment for haemochromatosis is called therapeutic venesection or phlebotomy (taking blood) with the aim of lowering your serum ferritin level. […] There is no cure for haemochromatosis, but treatments can keep the iron overload under control. This treatment is an effective and economical way of removing iron from the body. […] Therapeutic venesection in New Zealand is carried out in a variety of settings, including: hospital outpatient clinics, regional blood testing laboratories, the New Zealand Blood Service occasionally in some GP practices.

• #1 Living With Hemochromatosis | Hematology-Oncology Associates of CNY

  https://www.hoacny.com/patient-resources/blood-disorders/what-hemochromatosis/what-causes-hemochromatosis/living

  After the initial treatment period, you may need ongoing treatment two to six times a year. This will help prevent the iron from building up again. […] If you have hemochromatosis, getting ongoing care is important. Ongoing care may include: Continuing therapeutic phlebotomy, Taking medicines as prescribed, Contacting your doctor right away about new or worsening symptoms or possible reactions to phlebotomy, Following up regularly with your doctor about test results, ongoing treatments, and annual exams, Using a diary or log to track the amount of iron in your body. […] If you need routine phlebotomy, you may have to change your usual work times to schedule your treatments. You also may have to change your work schedule to allow for periods of fatigue (tiredness) or recovery, especially if your treatment weakens you.

• #1 Treatment – GPnotebook

  https://gpnotebook.com/en-AU/pages/haematology/haemochromatosis/treatment

  Dietary iron restriction is not necessary when undergoing phlebotomy. […] Iron and vitamin C supplementation should be avoided. […] Alcohol restriction is also advised. […] To minimize the risk of additional complications, patients with HFE-HC could be immunized against hepatitis A and B while iron overloaded. […] Vigorous physical activity should be avoided for 24h after phlebotomy. […] Iron chelation therapy can be used as a second line option in patients who are intolerant of phlebotomy. […] Deferoxamine or deferasirox is used. […] Clinical data on their use in hereditary haemochromatosis are limited.

• #1 Sign up for our monthly newsletter

  https://www.haemochromatosis.org.uk/genetic-haemochromatosis-care-pathway

  With treatment, many people live full and active lives with genetic haemochromatosis. […] For most people, treatment for genetic haemochromatosis is simple and effective; its a form of regular blood donation known as venesection. […] Initially, your doctor will ask you to attend hospital or a clinic weekly or fortnightly to have up to 1 unit (450 ml) of blood drawn. This has the effect of stimulating the body into producing more blood, which draws excess iron out of the organs and soft tissues. […] Venesection isn’t always an option for people (for example, if venous access is consistently troublesome); for a small number, alternative therapies including chelation therapy may be used instead (or interchangeably). These approaches use drugs administered orally or by injection which bind to excess iron to enable it to be passed out of the body.

• #2 Treatment of Hemochromatosis – NIDDK

  https://www.niddk.nih.gov/health-information/liver-disease/hemochromatosis/treatment

  In most cases, doctors treat hemochromatosis with phlebotomy, or drawing about a pint of blood at a time, on a regular schedule. This is the most direct and safe way to lower body stores of iron. […] Treatment of hemochromatosis can improve symptoms and prevent complications. […] Phlebotomy removes extra iron from your blood. Phlebotomy is simple, inexpensive, and safe. […] After phlebotomy has removed extra iron and blood levels of iron and ferritin return to normal, doctors will reduce phlebotomies to once every 1 to 3 months and eventually to 2 to 3 times a year. […] To treat secondary hemochromatosis in these people, doctors prescribe medicines, called chelating agents, that bind to iron and allow it to pass from the body in urine. […] Doctors treat neonatal hemochromatosis in newborns with exchange transfusions removing blood and replacing it with donor blood and IV immunoglobulin a solution of antibodies from healthy people.

• #2 Hemochromatosis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hemochromatosis/diagnosis-treatment/drc-20351448

  Medical professionals can treat hemochromatosis safely and effectively by removing blood from the body on a regular basis. This is similar to donating blood. The process is known as phlebotomy. […] The goal of phlebotomy is to lower the iron levels. The amount of blood removed and how often it’s removed depend on age, overall health and the severity of iron overload. […] Treating hemochromatosis can help relieve symptoms of tiredness, stomach pain and skin darkening. It can help prevent serious complications such as liver disease, heart disease and diabetes. If you already have one of these conditions, phlebotomy may slow the progression of the disease. In some cases, it may even reverse it. […] Phlebotomy can’t reverse cirrhosis or joint pain, but it can slow the progression. […] Phlebotomy may not be an option for someone who has certain conditions, such as anemia or heart complications. Instead, a healthcare professional may recommend a medicine to remove excess iron. The medicine can be injected into the body, or it can be taken as a pill. The medicine binds excess iron, allowing the body to expel iron through urine or stool in a process that’s called chelation (KEE-lay-shun). Chelation is not commonly used in hemochromatosis.

• #2 Treatment and Maintenance – Canadian Hemochromatosis Society

  https://www.toomuchiron.ca/hemochromatosis/treatment-and-maintenance/

  Treatment for hemochromatosis involves management of complications, screening for liver cancer, avoidance of supplemental iron and appropriate vaccinations for hepatitis A and B; however, an aggressive de-ironing protocol is most important. Excess iron is removed by a procedure known as phlebotomy, which is the drawing off of a unit of blood, using the same technique as a blood donation, but with a much higher frequency. […] This treatment is effective because phlebotomies remove red blood cells that contain iron. Each unit of blood contains approximately 225 mg of iron in hemoglobin, the main component of red blood cells. In the process of making new red blood cells, a signal is sent for the stored iron in the tissues and organs to be pulled out and transported to the bone marrow where red blood cells are produced. This repeated procedure gradually depletes the stores of excess iron and eventually the iron levels fall back to normal.

• #2 Patient education: Hereditary hemochromatosis (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/hereditary-hemochromatosis-beyond-the-basics

  In people with hemochromatosis who do not have symptoms at the time of diagnosis, the excess iron stores are removed after approximately 15 to 30 or fewer phlebotomy treatments. […] Maintenance phlebotomy — After excess iron has been removed, maintenance phlebotomy is used to prevent iron from reaccumulating. […] Effectiveness of phlebotomy — Phlebotomy can effectively remove iron and resolve some, but not all, complications of hemochromatosis. […] Chelation therapy (deferoxamine or deferasirox treatment) — Chelation therapy refers to treatment with a drug that removes iron from the body. […] Treatment of complications — Although phlebotomy can alleviate or even completely resolve some complications of hemochromatosis, other measures may be necessary to treat complications that persist.

• #2

  https://www2.hse.ie/conditions/haemochromatosis/treatment/

  There’s no cure for haemochromatosis. But there are treatments that can reduce the amount of iron in your body. […] Treatments can ease some of the symptoms. They can also reduce the risk of damage to organs such as your heart, liver and pancreas. […] The main treatment for managing haemochromatosis is venesection (phlebotomy). This is a procedure to remove some of your blood. It is like giving blood. […] There are 2 main stages to treatment: induction – blood is removed every week until your iron levels are normal […] maintenance – blood is removed about 2 to 4 times a year for the rest of your life.

• #2 Managing Your Hemochromatosis – Symptoms & Treatment | Carle.org

  https://carle.org/conditions/hemochromatosis

  The best therapy is removal of iron by taking blood (phlebotomy) once or twice weekly until the iron level is at or near normal. […] When phlebotomy is not possible or is insufficient and the disease is advanced, a medication called deferoxamine may also be given. It is known as an iron chelating agent and helps to remove iron from the body.

• #2 Treatment – GPnotebook

  https://gpnotebook.com/en-AU/pages/haematology/haemochromatosis/treatment

  Phlebotomy is the mainstay of treatment in patients with homozygous hereditary hemochromatosis and evidence of iron overload. […] The aim is to keep blood ferritin values 50-100 g/L. […] It is indicated in all patients with ferritin values 1000 g/L. […] Initially carried out once a week or fortnightly. […] With moderately raised ferritin levels (200-1000 g/L), initiation of phlebotomy depends on patient preference and clinical judgment. […] C282Y homozygotes without evidence for iron overload could be monitored annually and treatment instituted when the ferritin rises above normal. […] 400-500 mL is usually removed (which depletes 200-250 g of iron). […] The volume and frequency may be adjusted according to the degree of iron overload and baseline haemoglobin values. […] Haemoglobin and ferritin levels must be monitored regularly.

• #2 Treatment of Hemochromatosis – NIDDK

  https://www.niddk.nih.gov/health-information/liver-disease/hemochromatosis/treatment

  Phlebotomy can prevent the complications of hemochromatosis. […] Early treatment with phlebotomy can prevent complications of iron overload caused by these gene mutations. […] However, doctors can check iron levels and start treatment with chelating agents early, before iron overload causes damage to the liver, joints, and other organs.

• #2 Haemochromatosis

  https://dermnetnz.org/topics/haemochromatosis

  Treatment of haemochromatosis consists of regular removal of blood and is similar to making a blood donation (venesection therapy or phlebotomy). Every mL removed contains a 0.5 g of iron. Depending on the amount of iron overload the procedure may initially be performed once weekly or once monthly. This regularity of treatment continues until serum ferritin levels fall back to normal; this may take up to 2 years or more. After this, lifelong maintenance therapy needs to be set in place as excess iron continues to be absorbed. On average venesection is required every 34 months to prevent build-up and maintain healthy levels. Regular monitoring of serum ferritin, transferrin saturation, haematocrit and haemoglobin is necessary throughout the treatment process. […] It is essential to detect haemochromatosis as early as possible so that venesection can be instituted to prevent the build-up of iron and potential complications. Once complications such as diabetes and cirrhosis have developed, they cannot be reversed. Tiredness and abdominal pain should diminish, and increased pigmentation of the skin should fade over time once treatment has started. Arthritis, however, may fail to improve with venesection.

• #2 Hemochromatosis Treatment & Management: Approach Considerations, Surgical Intervention, Phlebotomy

  https://emedicine.medscape.com/article/177216-treatment

  Deferasirox (Exjade) is the oral iron chelator that should be taken once daily as an adjunct to phlebotomies or instead of phlebotomy in patients in whom these procedures are poorly tolerated. […] The first patient affected by juvenile hemochromatosis was successfully treated with chelation therapy. […] Patients affected with anemia cannot be treated with phlebotomy. Thus, application of iron chelation agents (eg, deferoxamine, deferiprone, deferasirox) is recommended. […] Dietary changes intended to minimize or eliminate iron ingestion are usually unnecessary and are often not feasible. […] Regular monitoring of hematocrit, hemoglobin, and serum ferritin levels is necessary in patients undergoing phlebotomy.

• #2 Hemochromatosis // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/hemochromatosis

  Phlebotomy may not be an option for someone who has certain conditions, such as anemia or heart complications. Instead, a healthcare professional may recommend a medicine to remove excess iron. The medicine can be injected into the body, or it can be taken as a pill. The medicine binds excess iron, allowing the body to expel iron through urine or stool in a process that’s called chelation (KEE-lay-shun). Chelation is not commonly used in hemochromatosis.

• #2 Hemochromatosis treatment 101

  https://www.singlecare.com/blog/hemochromatosis-treatment/

  Hemochromatosis is a treatable condition caused by excessive iron absorption, leading to potential organ damage and symptoms like joint pain, fatigue, and memory loss. […] Therapeutic treatments for hemochromatosis include phlebotomy (blood removal), chelation therapy (medication to bind and remove iron), and dietary adjustments to manage iron intake. […] Depending on the type and cause of your disorder, there are a few ways a healthcare provider may treat hemochromatosis. They include phlebotomy, chelation therapy, and diet and supplements. […] Therapeutic phlebotomy is a process of removing red blood cells, similar to donating blood, to lower iron levels. […] Regularly removing excess iron from the body helps prevent or reduce organ damage and manage symptoms associated with hemochromatosis.

• #2 Hemochromatosis (Iron Overload): Causes, Symptoms, Treatment, Diet & More

  https://my.clevelandclinic.org/health/diseases/14971-hemochromatosis-iron-overload

  Diet changes and other treatments can help ease the symptoms of hemochromatosis. They can also help prevent or delay further damage to your organs: […] Your healthcare provider will probably ask you to avoid supplements with iron. You may also need to stay away from foods with too much iron and limit vitamin C. Limit your alcohol consumption, too, because its not good for your liver. […] This medication removes extra iron from your body. Its taken by mouth at home or injected into the blood by a healthcare provider. […] This procedure uses a needle and tube to remove blood, and the iron it contains, from your body. Treatment needs to be repeated frequently, so you’ll have regular blood tests to measure iron levels. […] If another condition caused hemochromatosis, you may need treatment for that, too. And healthcare providers may recommend treating any problems caused by hemochromatosis.

• #2 Hemochromatosis treatment 101

  https://www.singlecare.com/blog/hemochromatosis-treatment/

  In some cases, your healthcare provider may recommend chelation therapy to help remove extra iron stored in the body. […] Dietary changes and supplements may be recommended by your healthcare provider if you are diagnosed with hemochromatosis. […] Watching what you eat can help you avoid additional complications. […] You may want to avoid red meat, which is high in iron, and excess alcohol, which can cause liver damage. […] Additionally, you should avoid iron and vitamin C supplements on their own or in multivitamins.

• #2 Treatment and Maintenance – Canadian Hemochromatosis Society

  https://www.toomuchiron.ca/hemochromatosis/treatment-and-maintenance/

  In the active de-ironing phase, reducing iron intake is almost insignificant compared to the 225 mg of iron that are being removed with each phlebotomy. In the maintenance phase, restricting iron intake may increase the time between phlebotomies but at no time will reducing iron in the diet replace phlebotomies as effective therapy.

• #2 Advancements in Hemochromatosis: A Comprehensive Overview of Clinical Trials

  https://www.lindushealth.com/blog/advancements-in-hemochromatosis-a-comprehensive-overview-of-clinical-trials

  While phlebotomy remains an essential treatment option, advancements in medical research have introduced alternative therapies, including iron chelation therapy and hepcidin mimetics. Iron chelation therapy involves the use of medications to bind and remove excess iron from the body, while hepcidin mimetics target the regulation of iron absorption. […] Iron chelation therapy, for example, has shown promising results in reducing iron levels in patients who cannot tolerate or do not respond well to phlebotomy. […] Moreover, hepcidin mimetics represent a groundbreaking approach to hemochromatosis treatment. By targeting the regulation of iron absorption, these medications offer a more targeted and precise method of managing iron overload. […] Novel drug therapies are currently being investigated as potential treatments for hemochromatosis. These include iron chelators with improved specificity and reduced side effects.

• #2 New treatment for iron overload on trial – UQ News – The University of Queensland, Australia

  https://www.uq.edu.au/news/article/2018/08/new-treatment-iron-overload-trial

  Liver expert Professor Darrell Crawford said haemochromatosis caused a build-up of iron in the body. […] The new treatment, on trial at The Gallipoli Medical Research Foundation at Brisbanes Greenslopes Private Hospital, aims to regulate iron absorption rather than remove excess iron by bloodletting. […] Haemochromatosis is currently treated by removing blood from the body three to four times a year, in a process similar to donating blood, Dr Crawford said. […] In our trial, patients will be injected once a week with a synthetic protein, hepcidin, to regulate iron absorption in the gut. […] Hepcidin is the master regulator of iron absorption and is deficient in patients with haemochromatosis. […] Early diagnosis and intervention restores life expectancy to normal for patients with haemochromatosis, Dr Crawford said.

• #2 Researchers find potential cure for deadly iron-overload disease | News Center

  https://med.stanford.edu/news/all-news/2020/09/researchers-find-potential-cure-for-deadly-iron-overload-disease.html

  Hemochromatosis can be treated by iron chelation therapy, but for many patients this is not an option due to concurrent kidney problems. In chelation therapy, drugs reduce excess levels of metals in the body to prevent toxicity. Chelation therapy is, unfortunately, toxic to the kidneys. Angelina’s liver disease had also caused kidney failure, so the chelation therapy wasn’t a safe option. That also is not uncommon. […] In a recently published study in Cell Reports, Rhee and her colleagues at the Stanford School of Medicine determined that a drug called ebselen may be able to prevent heart failure in patients with iron-overload disease. […] Finally, the researchers exposed these heart cells to a series of drugs, looking for an agent to reverse the iron-overload process. Ebselen proved to be the best candidate for blocking the channels for iron entry, potentially protecting against heart failure.

• #2 Hemochromatosis | Conditions & Treatments | UR Medicine

  https://www.urmc.rochester.edu/conditions-and-treatments/hemochromatosis

  Treatments include: […] Phlebotomy, which removes some blood from your body. This is done regularly at first until iron levels return to normal. Then it can be done once or twice a year as needed. […] Chelation therapy, using medicine to remove iron from your body. […] Stopping iron or vitamin C supplements. Vitamin C can increase the amount of iron you absorb. […] Limiting alcohol consumption […] Treatment of hemochromatosis complications, such as diabetes or heart or liver problems.

• #2 Haemochromatosis – British Liver Trust

  https://britishlivertrust.org.uk/information-and-support/liver-conditions/haemochromatosis/

  An enlarged liver may reduce in size but if cirrhosis has become advanced, improvement is unlikely. If you have cirrhosis, doctors may run blood tests and imaging tests at regular intervals (usually every six months). Having cirrhosis will put you at a much higher risk of developing hepatocellular carcinoma (HCC). If this occurs, a liver transplant may be required. […] In diabetes, phlebotomy will not be able to repair damage to your pancreas. Other serious problems such as arthritis and sexual disorders arising from damage to the pituitary gland are unfortunately unlikely to improve. Symptoms such as tiredness and abdominal pain should lessen with recovery. The colour of your skin should return to normal and if you have heart disease, such as cardiomyopathy, any improvement will be linked to the severity of any damage caused by haemochromatosis.

• #2 Hereditary Hemochromatosis – Hematology and Oncology – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/hematology-and-oncology/iron-overload/hereditary-hemochromatosis

  Treatment is usually with serial phlebotomies. […] Treatment is indicated for patients with clinical manifestations, elevated serum ferritin levels (particularly levels 1000 ng/mL [ 1000 mcg/L]), or elevated transferrin saturation 45%. […] Phlebotomy is the simplest and most effective method to remove excess iron. It delays progression of fibrosis to cirrhosis, sometimes even reversing cirrhotic changes, and prolongs survival, but it does not prevent hepatocellular carcinoma. […] Approximately 500 mL of blood (approximately 250 mg of iron) is removed weekly or every other week until serum ferritin levels reach 50 to 100 ng/mL (50 to 100 mcg/L). […] When iron levels are normal, phlebotomy can be done intermittently to maintain the ferritin level between 50 and 100 ng/mL. […] Diabetes mellitus, cardiomyopathy, erectile dysfunction, and other secondary manifestations are treated as indicated.

• #2 Hemochromatosis: Types, Risk Factors, and Causes

  https://www.healthline.com/health/hemochromatosis

  Early treatment and active management and monitoring of iron levels can help you avoid complications. […] The outlook for hemochromatosis varies. If a person receives treatment before organ damage has occurred, treatment can improve the outlook. […] Treatment can prevent further damage and may reverse any damage that is already present. With early treatment, there is a good chance of a normal lifespan.

• #2 Living With Hemochromatosis | Hematology-Oncology Associates of CNY

  https://www.hoacny.com/patient-resources/blood-disorders/what-hemochromatosis/what-causes-hemochromatosis/living

  The outlook for people who have hemochromatosis largely depends on how much organ damage has already occurred at the time of diagnosis. Early diagnosis and treatment of the disorder are important. […] Treatment may help prevent, delay, or sometimes reverse complications of the disorder. Treatment also may lead to higher energy levels and better quality of life. With early diagnosis and treatment, a normal lifespan is possible. […] If organ damage has already occurred, treatment may prevent further damage and improve life expectancy. However, treatment may not be able to reverse existing damage. […] If hemochromatosis isn’t treated, it can lead to severe organ damage or even death. […] At first, you may need to have therapeutic phlebotomy often. How long you’ll need this treatment depends on how much extra iron is in your body.

• #2 Haemochromatosis – Haemochromatosis Australia

  https://haemochromatosis.org.au/haemochromatosis/

  Many people with haemochromatosis can attend Lifeblood for venesection and they are able to use your donation to help save lives. If you are not eligible to donate blood which can be used to treat patients, Lifeblood may still be able to offer a therapeutic venesection service unless there are medical issues that would present a donor safety issue. […] Other options for venesection include: Many hospitals have regular venesection clinics, Some GPs will perform venesections in their surgery, Some pathology companies will perform venesections. It is usually best to ring and enquire first as not all collection centres provide this service.

• #2 Hemochromatosis | Conditions | UCSF Health

  https://www.ucsfhealth.org/conditions/hemochromatosis

  Treatment for hemochromatosis involves having a set amount of blood removed regularly. Over time, this lowers iron to normal levels and prevents conditions such as liver disease. Patients need to have periodic blood draws for life. […] The treatment for hemochromatosis is safe and simple. It involves removing the excess iron from the body by drawing your blood, usually a pint at a time in regular intervals. This is done in the same way in which blood is drawn from donors at blood banks. […] Once iron levels return to normal, you must give a pint of blood every two to four months for life. When the body replenishes the blood, it draws iron from storage sites such as the liver. Repeated over time, this process eventually eliminates the excess iron and risk of chronic liver disease. […] It is important to note that alcohol abuse contributes to the damage caused by excess iron in the liver and should be avoided. Also, people with cirrhosis from hemochromatosis are at increased risk of liver cancer and should have regular surveillance studies. Liver transplantation is an option for patients with liver failure or early cancer.

• #3 Haemochromatosis | Better Health Channel

  https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/haemochromatosis

  Treatment includes regularly removing blood until iron levels normalise. […] A person with haemochromatosis is treated with venesection. This is a procedure similar to blood donation, where around 500 mls of blood is removed until iron in the blood is reduced to normal levels. Depending on the severity of the condition, this may take around one and a half years of twice-weekly visits. Once iron levels are normal, venesection needs to be performed three or four times every year for life. If haemochromatosis is treated in its earliest stages before severe organ damage has occurred, there is no reduction in life expectancy other things being equal.

• #3 Hemochromatosis Treatment & Management: Approach Considerations, Surgical Intervention, Phlebotomy

  https://emedicine.medscape.com/article/177216-treatment

  Despite advances in the molecular understanding of hemochromatosis and the impact of C282Y on diagnosis, treatment remains simple, inexpensive, and safe. […] The goal of therapy in patients with iron overload disorders is to remove the iron before it can produce irreversible parenchymal damage. This is achieved via chelation therapy or venesection, depending on the underlying cause. […] A Cochrane database review of interventions for hereditary hemochromatosis found that phlebotomy remained the treatment of choice in those with hereditary hemochromatosis who required blood letting, but no data from randomized trials provided evidence of benefit from any form of blood letting in these patients. […] Once diagnosed, hemochromatosis is treated by phlebotomy to rid the body of excess iron and to maintain normal iron stores. Phlebotomy remains the sole recommended treatment for hereditary hemochromatosis and should be undertaken in a case-specific manner.

• #3 Iron overload – Wikipedia

  https://en.wikipedia.org/wiki/Iron_overload

  Phlebotomy, bloodletting or venesection is the mainstay of treatment in iron overload, consisting of regularly scheduled blood draws to remove red blood cells (and iron) from the body. Upon initial diagnosis of iron overload, the phlebotomies may be performed weekly or twice weekly, until iron levels are normalized. Once the serum ferritin and transferrin saturation are within the normal range, maintenance phlebotomies may be needed in some (depending upon the rate of reabsorption of iron), scheduled at varying frequencies to keep iron stores within normal range. A phlebotomy session typically draws between 450 and 500 mL of blood. Routine phlebotomy may reverse liver fibrosis and alleviate some symptoms of hemochromatosis, but chronic arthritis is usually not responsive to treatment. In those with hemochromatosis; the blood drawn during phlebotomy is safe to be donated.

• #3 Hemochromatosis | Cleveland Clinic

  https://my.clevelandclinic.org/departments/digestive/medical-professionals/hepatology/hemochromatosis

  The treatment of HH is simple and relatively safe. Therapeutic phlebotomy will effectively mobilize and remove iron stores and, when adhered to on a regular basis, will maintain them at normal levels. Patients should be encouraged to adhere to a regimen of phlebotomy of one unit of blood once or twice weekly as tolerated initially. This will remove approximately 250 mg of iron for each unit of phlebotomy, depending on the starting hematocrit value. In situations in which total body iron stores exceed 20 to 30 g, this regimen of phlebotomy may take up to 2 to 3 years to complete. The aim is to reduce iron stores to a level just short of iron deficiency. The hematocrit value should be monitored before each phlebotomy and should be postponed if it falls by more than 20% of its starting value. It is reasonable to check the serum ferritin level after every 10 to 12 phlebotomies. The serum ferritin level may be expected to fall progressively with iron mobilization, and it can be confidently assumed that effective mobilization of the iron stores will be completed when the serum ferritin level falls below 50 ng/mL.

• #3 Iron overload and iron chelation therapy – Leukaemia Foundation

  https://www.leukaemia.org.au/blood-cancer/journey/active-treatment/other-side-effects/iron-overload-and-iron-chelation-therapy/

  Iron chelation therapy is the treatment of iron overload. Iron chelation medications bind to the iron. This allows the body to remove the excess iron through urine and faeces. […] Iron chelating medications are: oral tablet – Deferasirox, subcutaneous or intravenous infusion – Desferrioxamine. […] Iron chelating therapy is usually ongoing, or until your iron levels are normal. Your treatment team will tell when to take iron chelating medication and for how long. […] It is important to note that iron chelation therapy is not suitable for all people. Talk to your treatment team about the possibility of iron overload.

• #3

  https://bpac.org.nz/bt/2015/april/haemochromatosis.aspx

  Patients undergoing venesection often experience improvement in subjective symptoms of lethargy and abdominal pain, and changes in skin pigmentation. […] However, venesection does not reverse all the characteristic symptoms and sequelae of iron overload: liver cirrhosis, arthropathy, testicular atrophy or thyroid dysfunction, and the symptoms patients experience as a result of these complications, usually do not improve with treatment. […] Given that haemochromatosis involves increased iron absorption in the gut, restricting dietary iron would appear at face value to be an intuitive treatment. However, there is limited evidence to support a change in diet. […] Patients with haemochromatosis should avoid dietary supplements containing iron, and also avoid supplements with vitamin C. […] A systematic review published in 2013 found that no randomised controlled trials had assessed dietary iron reduction and its effects on haemochromatosis management. […] Therefore, dietary iron reduction may reduce clinical burden but there is no data on the longer-term effects of dietary iron reduction on prognosis.

Zobacz więcej