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Hemosiderosis
Hemosiderosis is a form of iron overload disorder resulting in the accumulation of hemosiderin. Types include: * Transfusion hemosiderosis * Idiopathic pulmonary hemosiderosis * Transfusional diabetes Organs affected: * Hemosiderin deposition in the lungs is often seen after diffuse alveolar hemorrhage, which occurs in diseases such as Goodpasture's syndrome, granulomatosis with polyangiitis, and idiopathic pulmonary hemosiderosis. Mitral stenosis can also lead to pulmonary hemosiderosis. * Hemosiderin collects throughout the body in hemochromatosis. * Hemosiderin deposition in the liver is a common feature of hemochromatosis and is the cause of liver failure in the disease. * Selective iron deposition in the beta cells of pancreatic islets leads to diabetes due to the distribution of transferrin receptor on the beta cells of islets and in the skin leads to hyperpigmentation. * Hemosiderin deposition in the brain is seen after bleeds from any source, including chro ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Idiopathic Pulmonary Haemosiderosis
Idiopathic pulmonary haemosiderosis (IPH) is a lung disease of unknown cause that is characterized by alveolar capillary bleeding and accumulation of haemosiderin in the lungs. It is rare, with an incidence between 0.24 and 1.23 cases per million people. Pathophysiology Being idiopathic, IPH by definition has an unknown cause. It is thought to be an immune-mediated disease. The lung bleeding causes accumulation of iron, which in itself causes additional lung damage. Meanwhile, there is insufficient iron for inclusion into the haemoglobin molecules inside red blood cells which carry oxygen to body tissues for cellular respiration. Idiopathic pulmonary haemosiderosis can occur either as a primary lung disorder or as the sequela to other pulmonary, cardiovascular or immune system disorder. * PH1 involves PH with circulating anti-GBM antibodies. * PH2 involves PH with immune complex disease such as systemic lupus erythematosus, SLE. * PH3 involves no demonstrable immune system in ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Iron Overload
Iron overload is the abnormal and increased accumulation of total iron in the body, leading to organ damage. The primary mechanism of organ damage is oxidative stress, as elevated intracellular iron levels increase free radical formation via the Fenton reaction. Iron overload is often ''primary'' (i.e hereditary haemochromatosis, aceruloplasminemia) but may also be ''secondary'' to other causes (i.e. transfusional iron overload). Iron deposition most commonly occurs in the liver, pancreas, skin, heart, and joints. People with iron overload classically present with the triad of liver cirrhosis, secondary diabetes mellitus, and bronze skin. However, due to earlier detection nowadays, symptoms are often limited to general chronic malaise, arthralgia, and hepatomegaly. Signs and symptoms Organs most commonly affected by hemochromatosis include the liver, heart, and endocrine glands. Hemochromatosis may present with the following clinical syndromes: * liver: chronic liver di ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Transfusion Hemosiderosis
Transfusional hemosiderosis is the accumulation of iron in the body due to frequent blood transfusions. Iron accumulates in the liver and heart, but also endocrine organs. Frequent blood transfusions may be given to many patients, such as those with thalassemia, sickle cell disease, leukemia, aplastic anemia, or myelodysplastic syndrome, among others. It is diagnosed with a blood transferrin test and a liver biopsy. It is treated with venipuncture, erythrocytapheresis, and iron chelation therapy. Signs and symptoms Transfusional hemosiderosis can cause cardiac arrhythmia and cardiomyopathy. Causes Transfusional hemosiderosis is a potential side effect of frequent blood transfusions. These may be given for a number of conditions, including: * thalassemia. * sickle cell disease. * leukemia. * aplastic anemia. * myelodysplastic syndrome. Mechanism Hemoglobin, the oxygen-carrying molecule in a red blood cell, contains iron. The body has limited ways to store and remove iron. ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Superficial Siderosis
Superficial hemosiderosis of the central nervous system is a disease of the brain resulting from chronic iron deposition in neuronal tissues associated with cerebrospinal fluid. This occurs via the deposition of hemosiderin in neuronal tissue, and is associated with neuronal loss, gliosis, and demyelination of neuronal cells. This disease was first discovered in 1908 by R.C. Hamill after performing an autopsy. Detection of this disease was largely post-mortem until the advent of MRI technology, which made diagnosis far easier. Superficial siderosis is largely considered a rare disease, with less than 270 total reported cases in scientific literature as of 2006, and affects people of a wide range of ages with men being approximately three times more frequently affected than women. The number of reported cases of superficial siderosis has increased with advances in MRI technology, but it remains a rare disease. Symptoms Superficial siderosis is characterized by many symptoms resultin ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Siderosis
Siderosis is the deposition of excess iron in body tissue. When used without qualification, it usually refers to an environmental disease of the lung, also known more specifically as pulmonary siderosis or Welder's disease, which is a form of pneumoconiosis. Pulmonary siderosis was first described in 1936 from X-ray images of the lungs of arc welders. The name ''siderosis'' comes from Ancient Greek word for iron, ''sídēr(os)'', and has an -''osis'' suffix. Signs and symptoms Pulmonary siderosis does not usually cause harmful scar tissue formation within the lungs, which is why it said to be non-fibrotic condition, unlike asbestosis for example, and has also been called "benign pneumoconiosis". Mild to moderate scarring of the lungs has been found in unusual cases of pulmonary siderosis. These people have had persistent breathlessness, coughing and decreased lung function. However, people in occupations where they are exposed to iron (or rust) dust are usually also exposed ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Stasis Dermatitis
Stasis dermatitis refers to the skin changes that occur in the leg as a result of "stasis" or blood pooling from insufficient venous return; the alternative name of varicose eczema comes from a common cause of this being varicose veins. Insufficient venous return results in increased pressure in the capillaries with the result that both fluid and cells may "leak" out of the capillaries. This results in red cells breaking down, with iron containing hemosiderin possibly contributing to the pathology of this entity. Symptoms Stasis dermatitis may be characterized by: * Skin that appears thin, brown and tissue-like, with possible skin lesions ( macule or patches), red spots, superficial skin irritation and/or darkening and/or thickening of the skin at the ankles or legs * Weak skin may ulcerate in some areas and legs, ankles, or other areas may become swollen * Open sores, ulcers * Itching and/or leg pains * Sometimes pain may persist from swollen tissues and may feel like "stabbi ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Paroxysmal Nocturnal Hemoglobinuria
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, life-threatening disease of the blood characterized by destruction of red blood cells by the complement system, a part of the body's innate immune system. This destructive process occurs due to deficiency of the red blood cell surface protein DAF, which normally inhibits such immune reactions. Since the complement cascade attacks the red blood cells within the blood vessels of the circulatory system, the red blood cell destruction (hemolysis) is considered an ''intravascular'' hemolytic anemia. There is ongoing research into other key features of the disease, such as the high incidence of venous blood clot formation. Research suggests that PNH thrombosis (a blood clot) is caused by both the absence of GPI-anchored complement regulatory proteins (CD55 and CD59) on PNH platelets and the excessive consumption of nitric oxide (NO). PNH is the only hemolytic anemia caused by an ''acquired'' (rather than inherited) intr ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Blood Transfusion
Blood transfusion is the process of transferring blood products into a person's Circulatory system, circulation intravenously. Transfusions are used for various medical conditions to replace lost components of the blood. Early transfusions used whole blood, but modern medical practice commonly uses only components of the blood, such as red blood cells, blood plasma, plasma, platelets, and other clotting factors. White blood cells are transfused only in very rare circumstances, since granulocyte transfusion has limited applications. Whole blood has come back into use in the Major trauma, trauma setting. Red blood cells (RBC) contain hemoglobin and supply the Cell (biology), cells of the body with oxygen. White blood cells are not commonly used during transfusions, but they are part of the immune system and also fight infections. Plasma is the "yellowish" liquid part of blood, which acts as a buffer and contains proteins and other important substances needed for the body's overall ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
