In this disease, the bone marrow to produce red blood cells stops in the necessary amount: leukocytes, platelets and erythrocytes. Various types of diseases occur in different ways, some of them do not come immediately, but others appear only after a time.
What it is?
Aplastic anemia - is often acquired acute, subacute or chronic disease of the blood system, the basis of which is a violation of the hematopoietic function of bone marrow, and is a sharp decline in its ability to produce cells blood. It is a serious disease, leading even to death, fortunately, is rare. Likely to develop in men and women is about the same.
Causes and risk factors
Approximately half of the patients to establish the cause is not possible. In this case we speak of idiopathic (cryptogenic) aplastic anemia. To date, the following are known factors that trigger the disease development:
- exposure to ionizing radiation;
- immune diseases (symptom "graft versus host", eosinophilic fasciitis, thymoma and thymic carcinoma);
- accumulation in the tissues of salts of heavy metals (mercury, silver, copper, zinc, bismuth, lead);
- long production contacted with aromatic hydrocarbons (e.g., benzene), paint compositions, pesticides, organochlorine compounds;
- previously transferred infectious inflammatory diseases (influenza, hepatitis, cytomegalovirus infection, AIDS, sepsis, tuberculosis, brucellosis, leishmaniasis et al.);
- therapy of certain medications (headache tablets and derivatives pyrazolone series, chloramphenicol, sulfonamides, cytostatic agents, immunosuppressants, drugs of gold, antithyroid drugs, Analgin, indomethacin, anticonvulsants drugs).
The possible presence of predisposing genetic defect that leads to the development of aplastic anemia, indicates the fact increased incidence in patients with HLA-DR2 antigen, wherein the frequency of the DR antigen detection parents of patients above expected. In children with severe aplastic anemia showed a very significant increase in HLA-DPw3 antigen frequency.
Under the influence of these factors in the body starts a cascade of pathological reactions:
- immunosuppression;
- metabolic disorder and launching programmed death of blood stem cells;
- loss of hematopoietic stem cells, the source of the formation of all types of blood cells;
- damage to the microenvironment of hematopoietic stem cells, which leads to aggravation of the violation of its functions;
- shortening the life of red blood cells.
The pathogenesis of the disease
Aplastic anemia is caused by the suppression of blood cell production in the bone marrow of the process. Possible increased level of destruction and the destruction of the cell's own antigens. Studied the mechanism of apoptosis (spontaneous process destroys erythrocyte). It equates to a "programmed suicide."
- It found an increase in activity of the enzymes in the blood of a destructive action, the deficit of nucleic acids. The destruction of red blood cells observed in bone marrow maturation at all levels. Reduced lifespan of red blood cells. Since iron utilization is broken, excess deposited in the liver and spleen. Formed raising hormone erythropoietin, but the bone marrow does not respond to his orders.
- With the decline of granulocytes blocks their function is to participate in the immune defense of the body. A corresponding manifestation of reduced clotting occurs due to thrombotic syndrome.
The number of lymphocytes increased. Bone marrow becomes "empty" (panmieloftiz). Expressed oxygen deficiency in the tissue causes degenerative changes in internal organs. Particularly affected are the endocrine glands.
Classification
In addition to various etiological embodiment aplastic anemia (drug, postgepatitnogo, idiopathic), distinguish sharp (up to 1 month. flow), subacute (1 to 6 months.) and chronic (for more than 6 months.) form of the disease. Based on the severity of thrombosis and granulocytopenia aplastic anemia is divided into Grade 3:
- very severe (less 20,0h109 platelets / l; granulocyte less 0,2h109 / l)
- severe (less 20,0h109 platelets / l; granulocyte less 0,5h109 / l), according trepanobiopsy - low cellularity of bone marrow (less than 30% of normal)
- moderate (more 20,0h109 platelets / l; granulocyte more 0,5h109 / l)
Aplastic anemia occurring with selective inhibition of erythropoiesis, called partial red cell aplasia.
Symptoms of aplastic anemia
Severity of disease symptoms is proportional to the nature of reducing the amount of formed elements (pancytopenia).
Clinical symptoms of aplastic anemia are organized into three major syndromes:
- Anemic syndrome due to lower levels of red blood cells. His symptoms appear first, and include weakness, fatigue, decreased performance, wheezing, dizziness, tinnitus, tachycardia, decreased appetite, and sleep impairment, pale skin with a characteristic lemon flavor. Says poor tolerance stuffy room;
- Hemorrhagic: spontaneously occurring uterine, nasal, gingival and gastrointestinal bleeding, rashes on the specific skin and mucous membranes - of the type of point hemorrhages or bruises, bad blood stops at random cuts or on the fence analysis. Retinal hemorrhage can cause a patient's blindness.
- Leykotsitopenichesky syndrome that occurs in connection with a reduction in white blood cell count. This process affects the state of the body's defenses and is manifested in the form of frequent infections and colds that last a long time and are difficult to treat. There may be a rise in body temperature for no apparent reason.
In most cases, the disease develops gradually over several weeks or even months after exposure to a possible precipitating factor. However, it is possible acute onset of the disease.
possible complications
When aplastic anemia can cause the following:
- various bleeding - bleeding complications. The most dangerous complication - hemorrhagic stroke (cerebral certain portion impregnated with the blood, bringing the die);
- anemic coma - no reaction to what is happening because of the loss of consciousness, resulting from receipt of insufficient amounts of oxygen to the brain, which is caused by a significant decrease in the number of red blood cells;
- in the presence of chronic diseases deteriorating condition of internal organs.
- complications of infectious nature - the development of diseases which are caused by various microorganisms (bacteria, fungi, viruses);
If the disease is left untreated, 90% of patients die within a year.
Treatment for aplastic anemia
When diagnosed with aplastic anemia, treatment should begin as soon as possible, until the bone marrow is not completely stopped the production of blood cells. Patients with this diagnosis prescribe a blood transfusion, which allows you to compensate for the lack of blood cells in the body. However, it should be understood that such therapy is not a cure and only eliminates the symptoms, and has a supporting effect.
Be sure to make sure that the abnormality is not caused by certain adverse effects - if it is so unfavorable factors before treatment is necessary to eliminate.
- The most effective treatment of such pathology, as aplastic anemia is a bone marrow transplantation. This treatment is indicated even for those patients who have detected a severe form of disease. It is important to graft taken from a donor at five or more criteria matched bone marrow recipients. Otherwise possible graft rejection.
- In the treatment process it is also very important to protect against the possible penetration of bacteria and fungi, for which patients are prescribed the appropriate antifungal and antibacterial preparations.
patients receiving immunosuppressive drugs are also prescribed - these drugs disarm the immune system, not allowing it to destroy the cells of the brain that gives a chance to bone marrow re-start the process hematopoiesis. To eliminate the side effects from taking immunosuppressive drugs are often prescribed to people simultaneously taking steroids. And since the drugs are administered intravenously, and the procedure is controlled by the doctor, at this stage of hospitalization of the patient to the hospital.
Aplastic anemia: forecast
Forecast for life, disability and health status of patients with symptoms of aplastic anemia It depends on the severity of the disease and reducing the depth of vital cells hematopoiesis.
- Unfortunately, to this day, the world statistics show disappointing data on mortality from aplastic anemia, no matter how progressively evolving pharmaceutical industry in relation to the treatment of blood diseases. High mortality observed in patients with severe pancytopenia and to a greater extent the cause of death is a generalized form of sepsis, as the final step of leukopenia.
- Significantly improved predictions after initiation of radical therapies (transplantation of bone marrow stem cells, the use of immunosuppressive drugs). In 50-90% of cases the use of these methods of treatment can achieve complete recovery of the patient.
The use of anti-thymocyte and anti-lymphocyte antibodies in combination with cyclosporine therapy allows only 60-70% of cases, to achieve disease remission.
prevention
Clinical advice of doctors provide two options for prevention: primary and secondary.
Primary is designed for a relatively healthy person. To prevent the development of anemia, you need to eat right and feasible sports. We should not forget about the regular procedures to strengthen the immune system. It is also necessary to periodically undergo preventive examination by a physician, and the detection of ailments immediately start treatment.
Secondary prevention is performed to slow the progression of the disease has already been verified. For this purpose it is necessary to take prescription medications, carefully monitor the health and the appearance of new symptoms immediately seek medical help.
