Aplastic anemia - is often acquired acute, subacute or chronic disease of the blood system, which is based on a violation of hematopoietic function of bone marrow, namely, a sharp decline in its ability to produce blood cells.It is a serious disease, leading even to death, fortunately, is rare.Chance of sick men and women is about the same. Content
Unfortunately, the etiological factors of aplastic anemia today completely unknown.It is believed that the reason may be:
If the connection to the reasons mentioned above, can not be established, is considered idiopathic aplastic anemia.
It is known that the formation of blood provided by a special group of cells - hematopoietic stem cells.They have the ability to proliferate and differentiate into various hemopoietic stem from which subsequently form mature blood cell
defeat stem cells in this disease confirms the effectiveness of bone marrow transplantation by the sisters or brothers of HLA-compatible system or from identical twins.The transplanted bone marrow from identical twins do not take root in the body of the recipient, if the latter did not receive pre-course of drugs which depress the immune system.This fact confirms the immune nature of aplastic anemia.
Clinical manifestations of aplastic anemia are very typical.Debuts disease is usually acute.There have been 3 specific syndrome, testifying about the oppression of a germ hematopoiesis:
auscultation (listening) of the heart doctor will determine the presence of noise, palpation (palpation) and percussion (rapping) abdomen - enlargement of the liver and / or spleen.Disease occurs in different ways: in some cases, it progresses quickly, in a few months or even weeks, resulting in death, others - are long, with alternating periods of relapse and remission.
To suspect the disease will help to refine the most simple examination - blood count, which in varying degrees, will be reduced the number of blood cells - red blood cells and hemoglobin, white blood cells, platelets, and elevated erythrocyte sedimentation rate dramatically.The degree of reduction in the blood levels of certain elements is variable in different patients.Changes can exist in isolation or in parallel.There may be a predominance of one class over the other cells.To confirm the diagnosis requires a biopsy of the bone marrow: a special needle pierce the sternum or iliac bone penetrate the bone marrow and are taking on a study some of it.Microscopic when aplastic anemia is determined by the prevalence of the bone marrow cells of adipose tissue in which there are small specks of individual islands of hematopoietic cells.On the basis of the overall results of a blood test and myelogram experts proposed criteria to set the diagnosis of severe aplastic anemia:
Treatment of the disease involves three components: an etiological, pathogenetic and symptomatic.If you know the reasons that could cause anemia (eg, prolonged contact with the insecticide), you must immediately remove them - it is very likely that the function of normal hematopoiesis after.Because anemia is a mandatory feature of the disease leads to serious violations of microcirculation in organs and tissues and their oxygen starvation, you must strive to normalize this link: the patient is prescribed red blood cell transfusions, while seeking a hemoglobin value of 80 g / l.The leading cause of death for persons suffering from aplastic anemia, are bleeding.To correct thrombocytopenia, a patient must be assigned platelet concentrates - Platelets.In addition, in order not to provoke bleeding, patients are not recommended physical activity, prohibited use of aspirin and other anti-inflammatory drugs, women should take drugs that inhibit (suppress) ovulation.
decrease in the number of white blood cells and neutrophils - leukopenia and neutropenia - are the third hematological sign of aplastic anemia.To adjust the level of these elements in the blood by leukocyte transfusions slurry impossible as they circulate in the body just a few hours and then killed.In this situation, come to the fore prevention and early treatment of infectious diseases.For this purpose, can be assigned antibacterial (fluconazole, norfloxacin), antivirals (acyclovir), and antifungal agents.If the background of the said treatment of the patient's body temperature is not normal, it recommended drugs that stimulate the blood, such as amphotericin B. In the case where there is no effect of this drug, the patient can be assigned infusion of granulocytes.To restore the hematopoietic function of bone marrow used drugs several groups.
most effective treatment for idiopathic aplastic anemia is a BMT - bone marrow transplantation.Ideal - identical twin bone marrow of the patient.In this case, the survival rate is about 80-86%.The younger the recipient, the better his chances of 5-year survival after BMT.However, even after the complete restoration of function of hematopoiesis after 5 years may re-development of the disease.
forecast aplastic anemia depends on many factors, the main ones are:
In individuals younger than 30 years forecast is more favorable in the case of heavy and super-heavy form of the disease - a more pessimistic.If the patient receives only symptomatic therapy, the possibility to live one year is only 10%.When using pathogenetic therapy and especially BMT duration of life of persons suffering from aplastic anemia, increases many times.In some cases, full recovery is possible.