Hemochromatosis: Bloodletting

People may not know they have hemochromatosis since in the early stages they may not have any symptoms. In this stage, the only way the disorder can be detected is through routine blood tests and the doctor noticing elevated iron levels in the blood. Usually, the first and most common symptoms of hemochromatosis is joint pain and fatigue, but these are symptoms which occur in several other diseases and disorders, so the diagnosis is frequently held up or even overlooked. “Pain in the knuckles of the pointer and middle finger, collectively called ‘The Iron Fist,’ is the only sign or symptom specific to hemochromatosis. However, not everyone with HHC experiences the Iron Fist” (“WHAT IS HEMOCHROMATOSIS?” n.d.).

He explains that it benefits the immune system by keeping macrophages (protective blood cells) low on iron even though there is an excess in the liver and other areas. That way, when macrophages carry diseases to the lymphatic system to be killed, they can’t multiply and use the lymphatic system to seize the rest of the body. This is what enabled the survivors of the bubonic plague, tuberculosis, and many other disease to continue living. This may be why hemochromatosis is one of the most prevalent diseases in people of European descent. Once again, a recurring topic is bloodletting. Moalem gives us the background of the practice and explains why something so rudimentary would still be used today: there is a ton of iron in

Effects. Dr Moalem mentioned that it can lead to liver failure, diabetes, cancer, and many other complications. The gene was mostly found in people of Western European origin. It was first discovered by a research team in California even though the disease was around for a long time. About one in four or one in 3 of Western Europeans carry a copy of the gene, but only about 200 of the people of that origin have the actual disease. Studies have suggested that the disease first emerged with the Vikings and as the Vikings started to colonize the European coast. Equally important is how people treated the disease. Bloodletting was an extremely popular practice to temporarily treat hemochromatosis. Patients with the disease would remove blood from their body so their

A sudden worsening of anemia resulting from infection or enlargement of the spleen is a common reason for a transfusion. Multiple blood transfusions, however, might cause health problems because of the iron content in the blood. Iron overload, called hemosiderosis, can damage liver, heart, pancreas and other organs also leading to diseases such as diabetes mellitus. Iron chelation therapy should be started in patients with SCD receiving regular blood transfusions to reduce excess iron levels. Infections are treated with antibiotic medicines and sometimes blood transfusions. At the first sign of an infection, such as a fever, it is important to see a doctor right away as this may represent a medical emergency for people with SCD (Hurston 2018). Early treatment of infection can help prevent problems and even save

The option that is most frequently used to treat the disease is dialysis. Dialysis takes a person blood and passes it through an artificial dialyzer which removes toxins and extra fluids from the patient’s blood. In addition to the “Core curriculum for the dialysis technician: a comprehensive review of hemodialysis” dialysis can be done in centers or at the patient’s own home, most of the time it’s done in centers three times a week for four hours.

Blood transfusions are another option. This can increase the number of red blood cells that cause anemia and makes the body weak and it can fight off other complications from the disease. Gene therapy is a newer treatment that inserts a normal gene into the bone marrow of a person with sickle cell anemia hoping for a result of normal hemoglobin but this process is still in an experimental stage.

Severe DBA can be treated by blood transfusions from a compatible host. Blood transfusions help by replacing the defective blood with healthy blood. However, a constant supply is needed as the person will still continue to produce the mutated cells. Constant transfusions can lead to organ damage due to an excess of iron. To combat this, one can undergo a bone marrow transfusion. With a bone marrow transfusion from a healthy donor, the affected person will begin to produce healthy blood cells with the new marrow. If successful, the bone marrow transplant will not fix any of the birth defects a person with DBA is likely to have, but it will stop the progression of the disease and allow the person to live out their lives. In spite of positive results however, bone marrow transfusions can be very dangerous. For some, the possible side effects of a bone marrow transplant outweigh the possible positive effects. Another good way to treat DBA non-invasively is by using corticosteroids to treat the anemia in DBA. In a large study conducted, more than 80% showed signs of improvement because of the steroids. However, over time the effectiveness for some decreased while for others remained largely the same. There were also several adverse side effects that were associated with this treatment.

In moderate cases, blood transfusions may help restore blood cell counts; however, transfusions may become less effective over time. In severe cases, treatments such as bone marrow transplant and stem cell therapy may be required to restore the ability of bone marrow to produce blood cells. Such treatments are generally effective in younger patients, but older patients may also require the use of immunosuppressant drugs or drugs that stimulate the bone marrow. In cases related to environmental factors, pancytopenia may resolve on its own when the precipitating factor is removed or the underlying condition is

According to the American Society of Hematology, approximately 35 million people in the United States are over the age of 65, and almost 10 percent of this population is currently anemic. Anemia occurs when red blood cell counts drop, or when red blood cells do not contain enough hemoglobin. In chapter seven of Biology Today and Tomorrow without Psychology, we learned hemoglobin is an oxygen-binding protein in red blood cells that consists of four polypeptides (2-alpha globins & 2-beta globins). Each globin holds a cofactor called a heme, and each heme has an iron

Typically, when a human body has a sufficient amount of iron, it will reduce its intake of iron. However, within certain bodies, it is always under the impression that there isn’t enough. Thus, it continues to absorb more iron and this iron overload is the result of a disease called hemochromatosis. Generally, the excess iron builds up in the joints, liver, heart, and other organs which can lead to symptoms that include physical difficulty, joint pain, and an irregular heartbeat. However, the symptoms alone is not enough when it comes to diagnosing hemochromatosis. Therefore, taking part in blood, liver, and/or DNA testing will be more accurate. After diagnosing hemochromatosis, people can undergo treatments such as the medical procedure called bloodletting. By

As mutations cause a lack of β-globin expression, resulting in an inefficient erythropoiesus and early apooptosis of the red blood cell lineage, the majority of patients become lifelong dependent on blood transfusions needing transfusion about every 2-4 weeks in order to maintain a normal range of HbA (11-14 g/l). (Poggiali E, Cassinerio E, Zanaboni L, Cappellini

there are two available blood related treatments which may shorten the duration of the illness and improve the

Therapeutic phlebotomy (TP) is the usual and most effective method of treatment form hemochromatosis. Therapeutic phlebotomy is the extraction of one unit, or approximately 450 ml, at the frequency of at least once every two weeks. The goal of this treatment is to get the ferritin levels in the blood below 50 ng/ml and the transferrin saturation levels below 50%. If this treatment is begun in the early stages of hemochromatosis, then the damage to the joints and organs can be prevented. If the person already has liver disease, then therapeutic phlebotomy may improve the performance of the heart, diabetes, and skin

Blood letting was a common practice used throughout history as a 'cure' for everything from acne, to the plague, to bleeding, and everything in-between! Galen's four humors theory contributed to this idea greatly, with the thought that there were four substances in the body that had to be kept in balance to stay healthy. One of the four substances was blood; if there was too much or too little, they would ill. Blood letting is still used today to treat the blood disorder hemochromatosis, where patients have too much iron in their blood. Now though, phlebotomy is done under safer conditions by medical professionals who remove a portion of blood approximately equal to a blood donation, one pint.

Hematopoiesis is maintained by the renewal of multipotent hematopoietic stem cells that give rise to lineage committed cells. Hematopoietic stem cells are controlled in in the bone marrow, where they reside in a quiescent state (Baker 2014). Hematopoietic transcription factors play a crucial role in the proliferation and commitment of hematopoietic stem cells and during the differentiation into their mature progeny. According to the stochastic model of hematopoiesis, long term hematopoietic stem cells that undergo extensive self-renewal begin to differentiate into short term hematopoietic stem cells with limited self-renewing potential (Baker 2014). Short term hematopoietic stem cells further differentiate into multipotent progenitors that