Hemosiderosis: symptoms and treatment
Hemosiderosis is a violation of metabolic processes in the body, which is explained by the excessive content of a pigment called hemosiderin in tissue cells. This pigment is formed as a result of the breakdown of hemoglobin, which is affected by endogenous enzymes. Hemosiderin is involved in the delivery of certain chemicals to tissues. Certain disorders in the body lead to the development of a disease such as hemosiderosis, including: its excessive absorption in the intestine, disruptions in metabolic processes, too intensive process of destruction of red blood cells.
Hemosiderosis is not the only designation for this pathology. Also, the disease is called pigmentary hemorrhagic dermatosis, chronic pigmentary purpura.
Hemosiderosis can be local, affecting only the skin and lungs, or it can be generalized. In the latter case, the pigment begins to accumulate in excess in the liver, bone marrow, kidneys, sweat and salivary glands, as well as in other internal organs.
Moreover, regardless of the form of the disease, it manifests itself with the same symptoms, including: hemorrhagic skin rash (the rash has a reddish tint), hemoptysis, anemia, chronic fatigue syndrome. Most often, older men suffer from hemosiderosis. Children of this pathology are practically not affected.
It is very difficult to treat hemosiderosis, since this disease is not just a defect in appearance. Its causes lie in global disorders of metabolic processes in the body, which lead to a malfunction in the functioning of all internal organs.
Doctors of various profiles can detect and treat hemosiderosis: dermatologists, pulmonologists, immunologists, hematologists. In this case, the patient is prescribed hormonal steroid drugs, cytostatics, angioprotectors, vitamin-mineral complexes, plasmapheresis.
Content:
- Types of hemosiderosis
- Causes of hemosiderosis
- Symptoms of hemosiderosis
- Pulmonary hemosiderosis
- Skin hemosiderosis
- Hemosiderosis of internal organs
- Diagnosis of hemosiderosis
- Treatment of hemosiderosis
- Prevention of hemosiderosis
Types of hemosiderosis
With hemolysis (destruction of red blood cells and the release of hemoglobin from them) outside the vascular bed in any organ or hematoma, hemosiderosis develops. Excessive content of pigment in a certain part of the body does not lead to the fact that the tissues are damaged, but if they have already been subject to sclerotic changes, then the work of the organ will be disrupted.
Deposits of hemosiderin in tissues (idiopathic pulmonary hemosiderosis):
With intravascular hemolysis, general hemosiderosis develops, which is accompanied by massive deposits of hemosiderin in the internal organs. The cells of the liver and spleen are most often affected, but other internal organs can also be affected. They change their light to a darker one, since a lot of pigment accumulates in them. Most often, general hemosiderosis develops against the background of systemic lesions of the body.
There are also several other forms of hemosiderosis:
- Pulmonary essential.
- Hereditary.
- Dermatological. This form of hemosiderosis is represented by such pathologies as: Mayocchi's disease, ocher dermatitis, Guzhero-Blum's disease, Schamberg's disease.
- Hepatic.
- Idiopathic.
Causes of hemosiderosis
Until now, the reasons for the development of hemosiderosis have not been fully disclosed. Hemosiderosis is not a primary disease, but a secondary condition that develops against the background of disorders already existing in the body.
The following persons are at risk for the development of hemosiderosis:
- People with diseases of the blood and hematopoietic system (leukemia, hemolytic anemia).
- People with infectious diseases: sepsis, brucellosis, malaria, typhoid.
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People suffering from autoimmune pathologies, from disorders in the immune system.
- People with diseases of the vascular wall.
- Patients with vascular pathologies: with chronic venous insufficiency, with high blood pressure.
- People with intoxication of the body.
- Women in whose body the Rh-conflict develops.
Hemosiderosis can occur in people who receive frequent blood transfusions. Also at risk are people with a hereditary predisposition to this disorder. Increase the likelihood of developing hemosiderosis dermatological diseases, wounds and abrasions on the skin, hypothermia, taking a number of medications, as well as excessive intake of iron along with food.
Symptoms of hemosiderosis
The symptoms of hemosiderosis are largely determined by where the lesion is located. Pathology manifests itself unexpectedly for a person, but it develops smoothly.
If hemosiderosis affects the skin, then the rash can persist on it for several months or even years. In this case, a person suffers from itching, which can be very intense. The pigmentation is in the form of a spot with clear boundaries. The spots themselves are colored red; when pressed on the skin, they do not disappear.
With pulmonary hemosiderosis, the patient begins to pursue shortness of breath, which occurs even at rest. A person suffers from a wet cough, during which bloody sputum is released. The body temperature rises to high levels, the symptoms of respiratory failure increase, the liver and spleen increase in size, and signs of anemia appear. After a few days, relief comes, while the level of hemoglobin in the body returns to normal.
Pulmonary hemosiderosis
Pulmonary hemosiderosis is a disease with an unexplained etiology. It has a severe chronic course. In this case, the patient often has repeated hemorrhages in the alveoli, and erythrocytes disintegrate in the pulmonary parenchyma. In parallel, a significant amount of hemosiderin is released from them. All these pathological processes lead to the fact that the lungs begin to cope with their main function worse and worse.
Symptoms of pulmonary hemosiderosis in the acute stage of the disease:
- Cough with bloody expectoration.
- Pale skin.
- Hemorrhages in the eye sclera.
- Feeling of chronic fatigue.
- Dyspnea.
- Chest and joint pain.
- Increased body temperature.
- Heart palpitations.
- Hypotension.
- Enlargement of the spleen and liver in size.
When the acute stage of the disease is left behind, the person begins to feel satisfactory. At this time, he is able to do his job, lead a full-fledged lifestyle. However, as the disease progresses, relapses become more frequent, and the lull period becomes shorter.
If hemosiderosis has a severe course, then the patient develops cor pulmonale, pneumonia, pneumothorax. Even the death of a person is possible.
After opening, a number of patients show brown induration of the lungs, which is rarely diagnosed during a person's life. In the blood of such patients, autoantibodies are formed, which are an adhesion of antibodies and antigens. This leads to the development of an inflammatory reaction, which is localized in the lungs, since it is their tissue that becomes the target for autoantibody attacks. Small vessels penetrating the lung parenchyma expand. Erythrocytes are released from them, which disintegrate directly in the lung tissue, which leads to an excessive accumulation of hemosiderin.
Skin hemosiderosis
When the skin is damaged, rashes appear on them that have a dark color. They are formed due to the excessive accumulation of pigment in the skin cells, against the background of the destruction of the capillaries that penetrate its papillary layer.
The intensity of the color of the rashes can vary, the same applies to their size. Re-emerging rashes have a more saturated color, which is close to red. Rashes that have been on the skin for a long time, on the contrary, turn pale, becoming brown or yellowish. The spots are located on the arms and legs, on the hands and forearms. Their size can reach 3 cm. Also, nodules, plaques, papules and petichiae are often formed on the skin. Patients may indicate itching and burning of the skin.
In the capillaries that penetrate the dermis, pressure increases. This leads to the fact that plasma containing erythrocytes begins to leak through them. They are destroyed, which entails hemosiderin deposits. A CBC will show a decrease in platelet count and anemia.
Most often, the cutaneous form of hemosiderosis occurs in the form of orthostatic, eczema-like and itchy purpura, or in the form of Mallorcan disease.
Schamberg's disease
Schamberg's disease is a common autoimmune disorder that has a chronic course. At the same time, red rashes appear on the human body, resembling a trace from the injection performed. Immune complexes begin to be deposited in the vascular walls, which lead to the development of an autoimmune inflammatory process in the inner layers of the dermis. For this reason, the skin becomes covered with small hemorrhages. As hemosiderin accumulates in its papillary layer, the spots increase in size, acquire a brown color. Later they merge, forming large plaques bordered with a bright red rim.
As the disease progresses, the plaques merge with each other and begin to atrophy. At the same time, the general well-being of a person does not suffer.
In general, dermatological hemosiderosis responds well to treatment, which differs from hemosiderosis of other localizations. Patients recover quickly.
Hemosiderosis of internal organs
With pronounced intravascular hemolysis of erythrocytes, the patient develops generalized or systemic hemosiderosis. Internal organs suffer, their working capacity is disrupted, which affects the well-being of a person.
In liver hemosiderosis, pigment deposition occurs in hepatocytes. The disease can develop as an independent pathology, or how to be the result of other disorders in the body, and often the cause of hemosiderosis cannot be found out. At the same time, the liver increases in size, which becomes obvious even when you press the area where it is located. As the disease progresses, ascitic fluid accumulates in the abdominal cavity, blood pressure rises, the skin becomes yellow, the spleen increases in size, pigmentation areas appear on the arms, armpits and face. If there is no treatment, then the patient develops acidosis and falls into a coma.
With hemosiderosis of the kidneys, they are covered with brown granules. The disease proceeds as nephritis or nephrosis. A protein component is found in the urine, the level of lipids in the blood rises. A person notes swelling of the lower extremities, pain in the lumbar region, the desire to eat disappears, and dyspeptic disorders develop. In the absence of adequate and timely therapy, renal failure develops, which often becomes the cause of death.
Hemosiderosis of the liver (left) and kidneys (right):
In addition, the accumulation of hemosiderin can occur in the brain, spleen and other internal organs. All these conditions are associated with severe disturbances from the affected systems, which are often fatal.
Systemic hemosiderosis is a disease that is associated with a threat to human life.
Diagnosis of hemosiderosis
To clarify the diagnosis, a standard examination of the patient is not enough.
After its completion, it is necessary to assign the following studies:
- Blood sampling for a general analysis with the determination of serum iron and the total ability of blood to bind iron.
- Biopsy of organ tissues with subsequent histological examination of the material taken.
- Desferal test. This study allows you to detect hemosiderin in the urine for which the patient is injected with the drug Desferal. The injection is given intramuscularly.
- Microscopic examination of the patient's dermis in the presence of skin rashes.
Auxiliary research methods are:
- X-ray examination.
- CT and scintigraphy.
- Performing bronchoscopy.
- Performing spirometry.
- Examination of sputum under a microscope and its bacteriological culture.
Treatment of hemosiderosis
Hemosiderosis therapy requires the following recommendations:
- Compliance with a diet with the refusal of alcoholic beverages. It is imperative to remove all products from the menu that can provoke an allergic reaction.
- It is important to avoid hypothermia, injury, overheating of the body, as well as mental and physical stress.
- All diseases must be treated promptly.
- Chronic foci of infection must be eliminated.
- It is important to avoid using cosmetics that can cause allergies.
- All bad habits must be forgotten.
Drug therapy involves the use of the following medications:
- Reception and local application of hormonal steroid drugs, including: Prednisolone, Betamethasone, Dexamethasone.
- Taking drugs to reduce the inflammatory response: Indomethacin and Ibuprofen.
- Reception of antiplatelet agents: Aspirin, Cardiomagnet.
- Taking drugs with an immunosuppressive effect: Azathioprine, Cyclophosphamide.
- Reception of angioprotectors: Diosmin, Hesperidin.
- Reception of nootropics: Piracetam, Vinpocetine, Maxidol.
- Taking medications against allergies: Suprastin, Tavegil, Diazolin.
- Taking vitamins and minerals: vitamin C, rutin, calcium.
Also, the patient may be prescribed iron preparations, drugs to stop bleeding, bronchodilators. Oxygen therapy is possible. Also, patients are referred for hemosorption, cryoprecipitation, cryotherapy and plasmapheresis. Sometimes a blood transfusion or removal of the spleen is required.
Traditional medicine recommends using an infusion of hazel bark and mountain arnica, as well as a decoction of thick-leaved bergenia.
Prevention of hemosiderosis
Hemosiderosis is a disease that is often exacerbated. Provided that a person receives quality treatment, relapses will occur less often, but for this you need to observe preventive measures, including: proper nutrition, undergoing treatment in specialized sanatoriums, and maintaining a healthy lifestyle.
To prevent the development of hemosiderosis, you need to adhere to the following recommendations:
- Do not self-medicate infectious diseases, but seek medical attention in a timely manner.
- Maintain vascular health.
- Control blood pressure, monitor your weight and cholesterol.
- Minimize the risks of body intoxication.
The author of the article: Shutov Maxim Evgenievich | Hematologist
Education: In 2013 he graduated from the Kursk State Medical University and received a diploma "General Medicine". After 2 years, completed residency in the specialty "Oncology". In 2016 completed postgraduate studies at the National Medical and Surgical Center named after N. I. Pirogov.
