Hemosiderin is a pigment that accumulates in the blood due to the breakdown of red blood cells, which are blood cells that carry oxygen to tissues and internal organs. Their life span is 120 days, after which they disintegrate. Therefore, the destruction of red blood cells occurs in the normal state. But if too many blood cells break down, there is an excess accumulation of hemosiderin pigment in the blood. Read more about the causes, symptoms, features of the diagnosis and treatment of this pathology later in the article.
Causes of pathology
Hemosiderin is a substance that, when excessively accumulated in the body, causes the development of a disease called hemosiderosis. There are two groups of causes of this pathology: exogenous and endogenous. In the first case, the influence of external factors on the body is noted. In the second case, the disease develops due to a violation of the internal environment of the body.
To endogenousfactors that lead to increased hemosiderin deposition include:
- acute inflammatory diseases of infectious origin - malaria, brucellosis;
- toxic poisoning;
- effect of certain medications;
- excessive intake of iron into the body with drugs containing it ("Sorbifer", "M altofer");
- blood transfusion with an incompatible group or Rh factor.
Among exogenous factors, the greatest attention is paid to heredity. There are some genetic diseases in which there is an excessive deposition of hemosiderin in the brain, liver and other internal organs. These are, first of all, such pathologies:
- thalassemia - impaired synthesis of one of the hemoglobin chains;
- sickle cell anemia - a congenital disorder of the shape of red blood cells;
- enzymopathies - a group of diseases in which there is not enough of any enzyme to form hemoglobin;
- membranopathy - congenital disorders of the structure of red blood cells.
Separately distinguish autoimmune diseases as the cause of the development of hemosiderosis.
Disease forms
Hemosiderin is a substance that can accumulate both everywhere in the body, in almost all internal organs, and in isolation, that is, in a particular place. In the first case, they speak of a generalized, or general form of the disease. In the second case, local, or local, hemosiderosis develops.
The emergence of the commonhemosiderosis occurs against the background of any systemic pathology. Then hemosiderin accumulates in the brain, liver and other organs. In the local form, the pigment is collected in localized areas of the human body. For example, in the cavity of a tubular organ or in a hematoma.
Depending on the cause of development, two more groups of the disease are distinguished:
- primary - the reasons for this form have not yet been clarified;
- secondary - develops against the background of other diseases.
The main causes of secondary hemosiderosis can be the following pathological conditions:
- leukemia - a malignant lesion of the bone marrow;
- cirrhosis of the liver;
- infectious diseases;
- skin diseases: pyoderma, eczema, dermatitis;
- hypertension with severe course;
- frequent blood transfusions;
- hemolytic anemia.
In fact, there are many more reasons for the development of secondary hemosiderosis, so only the main ones are presented in the previous section and above.
Risk factors for disease development
Separately, factors are isolated that do not directly lead to an increased deposition of hemosiderin, but increase the risk of this pathological condition. These include:
- permanent hypothermia of the body;
- chronic stress;
- excessive exercise;
- uncontrolled intake of diuretics, paracetamol, some antibiotics.
What organs are affected by hemosiderosis?
Hemosiderinis a pigment that can accumulate in almost any internal organ. But most often there is a defeat:
- liver;
- kidney;
- spleen;
- skin;
- bone marrow;
- salivary or sweat glands;
- brain.
Hemosiderosis of the skin: manifestations
The most striking manifestation is the accumulation of hemosiderin in the skin. In almost all patients, the main symptom is the formation of dark brown spots on the legs. Usually areas of pigmentation have a large diameter, but sometimes there are small, almost punctate rashes. Some patients develop a hemorrhagic rash, which is caused by damage to the capillaries of the skin.
The hue of the rash can vary from brick red to dark brown or yellow. In addition to spots, other elements of the rash appear: nodules, papules, plaques. The patient is concerned about the itching of the affected areas of the skin.
Hemosiderosis of the liver: symptoms
The deposition of hemosiderin in the liver tissue is manifested, first of all, by an increase in the size of the organ. This leads to stretching of the capsule surrounding the liver. The patient feels it as a dull pain on the right under the rib. With a significant increase, there is an asymmetry of the abdomen and its bulging on the right. Palpation of the abdomen in these areas is also painful.
With a long-term process, the function of the liver is gradually impaired. This is manifested by an increase in the abdomen due to the accumulation of fluid in it,varicose veins of the stomach and esophagus, hemorrhoidal veins, yellowing of the skin and sclera, hemorrhagic rashes.
Hemosiderosis of the kidneys: symptoms
The accumulation of pigment in the kidneys not only leads to changes in the urine, but also to certain clinical manifestations. Hemosiderin affects the renal tubules and glomeruli, which leads to impaired blood filtration and the release of protein and carbohydrates from it. As a result, hypoproteinemia develops - a decrease in the concentration of protein in the blood.
The patient complains of edema. First they appear on the face, and in advanced cases cover the entire body. The patient is concerned about general weakness and fatigue.
Prolonged damage to kidney function leads to dysfunction of other organs and systems.
Brain damage
Deposition of hemosiderin in the brain has very variable clinical manifestations. It all depends on in which particular department the lesion is localized.
The accumulation of hemosiderin causes the death of nerve cells, the destruction of the myelin sheath of nerves. Often, patients with hemosiderosis of the brain have a previous hemorrhage in the parenchyma, removal of tumors, hemorrhagic strokes.
Typical clinical manifestations of hemosiderin accumulation in the brain are:
- balance disorder - ataxia;
- hearing loss by type of sensorineural hearing loss;
- mental disorders;
- dysarthria - impaired speech;
- motion disorders.
Diagnosis
Diagnosis of hemosiderosis should be complex. Often, the coordinated work of doctors of various specializations is necessary: a dermatologist, a neurologist, a pulmonologist, an infectious disease specialist, and others. It all depends on which organ is predominantly affected.
Diagnostic search begins with a detailed questioning of the patient about his complaints, their development in dynamics, the presence of previous diseases. Only after that, additional examination methods are prescribed.
Regardless of the form of the disease, the following diagnostic methods are prescribed:
- Complete blood count - a reduced number of red blood cells and hemoglobin is determined.
- Determination of the presence of hemosiderin in the urine.
- Determine the level of iron in the blood serum.
- Analysis of the binding capacity of iron in the body.
- Biopsy of the affected tissue with histological examination to detect hemosiderin deposits.
Only a histological examination of the biopsy can make a diagnosis of hemosiderosis with absolute certainty. When examining a piece of tissue under a microscope, macrophages with hemosiderin are found, since it is these cells that are the first to "eat" the extra pigment.
Also, depending on the lesion of which organ the doctor suspects, he prescribes the following examination methods:
- magnetic resonance imaging of the brain;
- CT scan;
- ultrasound;
- radiography;
- bronchoscopy.
One morean effective laboratory diagnostic method is the desferal test. For its implementation, the patient is administered 500 mg of Desferal. A minimum of 6 hours and a maximum of 24 hours after the injection of the drug, the patient's urine is collected and the amount of iron in it is examined.
Treatment of disease
Since at the present stage the most attention is paid to the autoimmune course of hemosiderosis, drugs from the group of corticosteroids are considered priority drugs. They depress the immune system, thereby reducing the production of antibodies against their own red blood cells. These drugs include "Dexamethasone", "Prednisolone". But glucocorticoids help only in 40-50% of cases. In the absence of their effectiveness, the patient is prescribed cytostatics ("Methotrexate", "Azathioprine").
Also prescribed drugs that improve tissue trophism, cell metabolism, increasing the supply of oxygen to them. These drugs include:
- Venotonics. They increase the elasticity of the vascular walls, improve blood flow in the tissues of the brain - "Detralex", "Doppelhertz".
- Vitamins of group B. Improve the conductivity of the nerve impulse, the condition of the cord tissue.
- Vitamin C. Increases the strength of the vascular wall.
- Angioprotectors. They have a similar effect to vitamin C - "Etamzilat", "Vincamine".
- Nootropics. Improve blood flow in the brain and accelerate cell metabolism - Cerebrolysin, Phenibut.
- Neuroleptics. They are prescribed symptomatically only if the patient has mental disorders - "Aminazine".
In case of hemosiderosis of the kidneys and a significant impairment of their function, plasmapheresis or hemodialysis is prescribed.
Thus, excessive accumulation of hemosiderin is a serious pathological condition. It requires the earliest possible diagnosis and timely treatment, since in advanced cases, hemosiderosis leads to severe dysfunction of the internal organs. Often these damages are irreversible.
