Hemosiderosis
The content of the article:
- Causes and risk factors
- Forms of the disease
- Symptoms
- Diagnostics
- Treatment
- Possible complications and consequences
- Forecast
- Prevention
Hemosiderosis is a disease belonging to the group of pigmented dystrophies. It is characterized by the accumulation in the tissues of the body of an excess amount of hemosiderin, an iron-containing pigment.
Cutaneous manifestations of hemosiderosis
Causes and risk factors
Hemosiderin is formed during the breakdown of hemoglobin molecules by certain enzymes in sideroblast cells. Normally, a small amount of hemosiderin is found in cells of lymphoid tissue, bone marrow, spleen and liver. With an increase in the synthesis of hemosiderin, its excess is deposited in the cells of other tissues.
General hemosiderosis is always a secondary pathology, the development of which is caused by autoimmune processes, intoxication, infections or blood diseases. The most common causes of hemosiderosis are:
- hemolytic anemia;
- poisoning with hemolytic poison (quinine, lead, sulfonamides);
- leukemia;
- multiple blood transfusions;
- cirrhosis of the liver;
- rhesus conflict;
- infectious diseases (relapsing fever, malaria, brucellosis, sepsis);
- decompensated heart failure;
- chronic venous insufficiency;
- diabetes;
- dermatitis;
- eczema.
Some experts believe that hereditary predisposition and disorders of the immune system play an important role in the pathological mechanism of hemosiderosis.
Forms of the disease
According to the degree of spread of the pathological process, there are:
- general (generalized) hemosiderosis - it leads to intravascular (endovascular) hemolysis of erythrocytes arising against the background of any systemic processes. Hemosiderin is deposited in the spleen, red bone marrow, liver, as a result of which they acquire a rusty, brown color;
- local (local) hemosiderosis - develops as a result of extravascular (extravascular) hemolysis. The accumulation of hemosiderin can be observed both in small areas of the body (in the area of hematomas) and within an organ (for example, the lung).
Hemosiderin granules
According to the characteristics of the clinical course, there are:
- hemosiderosis of the skin (ocher dermatitis, purple angiodermatitis, lichenoid pigment dermatitis, senile hemosiderosis, ring-shaped telangiectatic purpura of Mayocchi, Shamberg's disease);
- pulmonary idiopathic hemosiderosis (brown induration of the lungs).
In some cases, the deposition of hemosiderin becomes the cause of dysfunction of the affected organ and the development of structural changes in it. This form of hemosiderosis is called hemochromatosis.
Symptoms
The clinical picture of hemosiderosis depends on the form of the disease.
Pulmonary idiopathic hemosiderosis is diagnosed mainly in children and young people, characterized by recurrent pulmonary hemorrhages of varying severity, increasing respiratory failure and persistent hypochromic anemia.
In the acute phase of the disease, there are:
- dyspnea;
- chest pain;
- cough with bloody sputum;
- pallor of the skin;
- icterus sclera;
- cyanosis of the nasolabial triangle;
- weakness;
- dizziness;
- increased body temperature.
Coughing up blood is one of the symptoms of hemosiderosis
During the period of remission, the clinical manifestations of the disease are very weak or completely absent. After each period of exacerbation of idiopathic pulmonary hemosiderosis, the duration of the remission period decreases.
The cutaneous form of hemosiderosis occurs more often in men over 40 years of age. The course is chronic, benign. Brick-red pigment spots appear on the skin of the forearms, hands, legs and ankles. Over time, the color of the elements of the rash changes to yellowish, dark brown or brown. Damage to internal organs in the cutaneous form of hemosiderosis does not occur, the general condition does not suffer.
Diagnostics
Diagnosis of hemosiderosis is based on the characteristic clinical signs of the disease, data from laboratory and instrumental examination methods. A general blood test is prescribed, the concentration of serum iron is determined.
If hemosiderosis is suspected, a desferal test is performed. To do this, deferoxamine is injected intramuscularly, and then the iron content in the urine is determined. The sample is considered positive if the urine contains more than 1 mg of iron.
Diagnosis of hemosiderosis involves a desferal test with the introduction of deferoxamine intramuscularly
To confirm the diagnosis, a puncture biopsy of the bone marrow, lung, liver or skin is performed, followed by a histological examination of the tissue obtained.
With hemosiderosis of the lungs, they also carry out:
- spirometry;
- microscopic examination of sputum;
- perfusion lung scintigraphy;
- chest x-ray;
- bronchoscopy.
Treatment
Therapy for pulmonary hemosiderosis begins with the administration of corticosteroids. If it is ineffective, patients are prescribed immunosuppressants and plasmapheresis sessions. In some cases, it is possible to achieve a stable remission after removing the spleen (splenectomy). In addition, symptomatic therapy is carried out with the appointment of bronchodilators, hemostatics and other drugs (depending on the manifestations of the disease).
As part of the complex treatment of hemosiderosis, plasmapheresis is used
Treatment of cutaneous hemosiderosis consists of topical application of corticosteroid ointments. Also, angioprotectors, calcium preparations, rutin, ascorbic acid are prescribed. In severe disease, the appointment of deferoxamine, PUVA therapy is indicated.
Possible complications and consequences
The most common complications of pulmonary hemosiderosis:
- recurrent pneumothorax;
- pulmonary bleeding;
- lung infarction;
- expansion and hypertrophy of the right heart;
- pulmonary hypertension;
- chronic respiratory failure.
Forecast
The prognosis for pulmonary hemosiderosis is serious. The disease progresses and leads to disability of patients. It can be complicated by massive pulmonary bleeding, leading to rapid death.
With the cutaneous form of hemosiderosis, the prognosis is favorable.
Prevention
Prevention of violations of the metabolic processes of hemosiderin consists in the timely treatment of hematological, vascular and infectious diseases, prevention of chemical or drug intoxication, blood transfusion complications.
Elena Minkina Doctor anesthesiologist-resuscitator About the author
Education: graduated from the Tashkent State Medical Institute, specializing in general medicine in 1991. Repeatedly passed refresher courses.
Work experience: anesthesiologist-resuscitator of the city maternity complex, resuscitator of the hemodialysis department.
The information is generalized and provided for informational purposes only. At the first sign of illness, see your doctor. Self-medication is hazardous to health!
