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 expectancy is 120 days, after which they break up. Therefore, the destruction of red blood cells occurs in the normal state. But if too many blood cells break up, there is an excessive accumulation of the 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 accumulated excessively 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 effect 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.
Endogenous factors that lead to increased deposition of hemosiderin include:
- acute inflammatory diseases of infectious origin - malaria, brucellosis;
- toxic poisoning;
- the effect of certain medications;
- excessive intake of iron in the body with drugs containing it (Sorbifer, Maltofer);
- 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 excessive deposition of hemosiderin occurs in the brain, liver, and other internal organs. These are, first of all, such pathologies:
- thalassemia - a violation of the synthesis of one of the hemoglobin chains;
- sickle cell anemia - a congenital disorder of the form of red blood cells;
- Enzymopathies - a group of diseases in which there is not enough enzyme for the formation of hemoglobin;
- membranopathy - congenital disorders of the structure of red blood cells.
Autoimmune diseases are singled out separately as a cause of hemosiderosis.
Forms of the disease
Hemosiderin is a substance that can accumulate both everywhere in the body, in almost all internal organs, and in isolation, that is, in some 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 occurrence of general hemosiderosis occurs against the background of any systemic pathology. Then hemosiderin accumulates in the brain, liver and other organs. In 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 following pathological conditions may be the main causes of secondary hemosiderosis:
- leukemia - a malignant lesion of the bone marrow;
- cirrhosis of the liver;
- infectious diseases;
- skin diseases: pyoderma, eczema, dermatitis;
- severe hypertension;
- 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
Separate factors that do not directly lead to increased deposition of hemosiderin, but increase the risk of this pathological condition. These include:
- constant hypothermia of the body;
- chronic stress;
- excessive physical activity;
- uncontrolled intake of diuretics, paracetamol, some antibiotics.
What organs are affected by hemosiderosis?
Hemosiderin is a pigment that can accumulate in almost any internal organ. But most often, a defeat is observed:
- liver
- kidney
- spleen;
- skin integument;
- bone marrow;
- salivary or sweat glands;
- brain.
Hemosiderosis of the skin: manifestations
The most striking manifestations are the accumulation of hemosiderin in the skin. In almost all patients, the main symptom is the formation of dark brown spots on the legs. Typically, pigmentation sites have a large diameter, but sometimes small, almost point rashes are found. In some patients, a hemorrhagic rash occurs , which is caused by damage to the skin capillaries.
The hue of the rashes can be different: from the color of red brick 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 this as a dull pain on the right under the rib. With a significant increase, asymmetry of the abdomen occurs and its bulging to the right. Palpation of the abdomen in these sections is also painful.
With a long ongoing process, liver function 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.
Kidney hemosiderosis: 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 the appearance of edema. First, they occur on the face, and in advanced cases cover the entire body. The patient is worried about general weakness and fatigue.
Long-term damage to kidney function leads to impaired functions of other organs and systems.
Brain damage
The deposition of hemosiderin in the brain has very variable clinical manifestations. It all depends on in which 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 cerebral hemosiderosis have previous hemorrhage to the parenchyma, removal of tumors, hemorrhagic strokes.
Typical clinical manifestations of hemosiderin accumulation in the brain are:
- imbalance - ataxia;
- hearing impairment by type of sensorineural hearing loss;
- mental disorders;
- dysarthria - speech impairment;
- motor disorders.
Diagnostics
Diagnosis of hemosiderosis should be comprehensive. The coordinated work of doctors of different specializations is often necessary: a dermatologist, a neurologist, a pulmonologist, an infectious disease specialist and others. It all depends on which organ is predominantly affected.
The diagnostic search begins with a detailed questioning of the patient about his complaints, their development in dynamics, the presence of previous diseases. Only after this additional methods of examination are prescribed.
Regardless of the form of the disease, the following diagnostic methods are prescribed:
- General blood test - 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 it possible to diagnose 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 excess pigment.
Also, depending on the defeat of which organ the doctor suspects, he prescribes the following examination methods:
- magnetic resonance imaging of the brain;
- computed tomogram;
- ultrasonography;
- roentgenography;
- bronchoscopy.
Another effective laboratory diagnostic method is a desferal test. For its implementation, the patient is administered 500 mg of desferal. A minimum of 6 hours and a maximum of one day after injection of the drug collects the patient’s urine and examines the amount of iron in it.
Disease treatment
Since at the present stage the greatest attention is paid to the autoimmune course of hemosiderosis, the drugs from the corticosteroid group are considered priority drugs. They inhibit the immune system, thereby reducing the production of antibodies against their own red blood cells. Such funds include Dexamethasone, Prednisone. But glucocorticoids help only in 40-50% of cases. In the absence of their effectiveness, the patient is prescribed cytostatics (Methotrexate, Azathioprine).
Also, drugs are prescribed that improve trophic tissue, cell metabolism, increasing the flow of oxygen to them. These drugs include:
- Venotonic. They increase the elasticity of the vascular walls, improve the blood flow in the brain tissue - "Detralex", "Doppelherz".
- Vitamins of group B. Improve the conductivity of the nerve impulse, the state of the nervous tissue.
- Vitamin C. Increases the strength of the vascular wall.
- Angioprotectors. They have a vitamin C-like effect - Ethamsylate, Vincamine.
- Nootropics. They improve blood flow in the brain and accelerate cell metabolism - Cerebrolysin, Phenibut.
- Antipsychotics. Appointed symptomatically only in the presence of mental disorders in the patient - "Aminazine".
With renal hemosiderosis and a significant violation 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 violations of the functions of internal organs. Often these violations are irreversible.