The concept of aplastic anemia
Aplastic anemia - a blood disorder,is in violation of the bone marrow's ability to produce three main types of blood cells. For the first time the disease was described by Paul Ehrlich in 1888. The term "aplastic anemia" was proposed in the early XX century (from the word "aplasia", meaning the absence of something or underdevelopment).
Aplastic anemia is a rare disease that is more common in young adults, with equal frequency in men and women.
When there is a decrease of aplastic anemiathe number of white blood cells (leukopenia) - this is the first component of the disease and the second component - the lack of platelets (thrombocytopenia), which leads to an increased risk of infection or bleeding, which are the main causes of death in children with aplastic anemia. There is a third component of - anemia caused by deficiency of red blood cells.
Causes of aplastic anemia
Among the main causes of aplasticanemia plays a big role hypersensitivity to drug formulation (idiosyncrasy). The most common cause aplastic anemia chloramphenicol (chloramphenicol), sulfonamides, tetracycline, streptomycin, phenylbutazone, gold compounds, barbiturates, bucarban, dekaris, antithyroid and antihistamines.
Among the physical factors necessary to allocateexposure to ionizing radiation. It recorded an increase in the incidence of aplastic anemia in patients receiving radiation therapy for diseases of the osteoarticular apparatus, as well as physicians, Radiologists.
In some patients with aplastic anemia beginningdiseases associated with infectious diseases such as viral hepatitis (A, B and C). Furthermore hepatitis, aplastic anemia can cause Epstein-Barr virus, herpes virus, parvavirusy and human immunodeficiency virus (HIV).
Symptoms of aplastic anemia
To distinguish two main groups of aplastic anemia:
- inherent (constitutional Fanconi anemia)
- acquired as a result of the impact of external factors
Early symptoms of aplastic anemia includegeneral weakness, fatigue, dizziness, tinnitus, poor tolerance of a stuffy room, the occurrence of bleeding (nasal, uterine, gastrointestinal, bleeding in the skin), unmotivated bruises, pain in bones and joints. Gradually growing pallor of the skin and mucous membranes. At congenital forms of aplastic anemia it has a characteristic skin ashen. Liver increased slightly. The spleen and peripheral lymph nodes were not enlarged.
In children with aplastic anemia increases depression of bone marrow function.
Aplastic anemia is usually characterized bysevere or mild. In less severe aplastic anemia, red blood cell count decreased not so much. Sometimes the lack of one of the types of blood cells prevails, causing heavy for aplastic anemia.
Often a mild form of aplastic anemiaIt develops into a severe form. When the disease of moderate severity are periodically celebrated bleeding from the nose, sometimes not heavy infection and mild anemia. In severe disease the patient is constantly suffering from severe, prolonged bleeding, frequent or severe infections, severe anemia.
The most common and most severeform of aplastic anemia - Fanconi anemia. Children with Fanconi anemia often have a kidney or bone defects. Malformations of the thumb and wrist bones are the most common. Malformations of the kidneys may affect one kidney, the kidney can occur more or improper placement of the kidneys.
Treatment of the disease
The treatment provides only temporary controldisease. However, among young children with severe disease, who receive the recommended form of treatment (bone marrow transplantation), four out of five survive and are considered completely cured.
Treatment for aplastic anemia isa very difficult task. The first and most important step in treatment is to eliminate all the factors that could cause anemia. The second step is the careful use of blood products. However, the main and the only pathogenetic treatment of aplastic anemia, can count on saving the patient's life is the transplantation of bone marrow from a compatible donor, conducted in special transplant centers. Transplanting should be done as soon as possible after diagnosis. No bone marrow transplantation and disease progresses rapidly over several months leads to death.
Children with aplastic anemia who are notperformed bone marrow transplantation, can not be completely cured, although a lasting improvement, as long as the normal number of blood cells. Patients with apparent recovery from aplastic anemia in a few years there may be a recurrence of the disease. Even while improving the number of blood cells is below normal, however, for several decades the degree of reduction can be above dangerous levels. The frequency of leukemia in patients with aplastic anemia is higher than in patients without aplastic anemia.
If it is impossible to choose a donor asreference drug used immunosuppressant cyclosporine A. In patients with non-severe aplastic anemia using this drug allows you to calculate in some cases of success. Currently, major research centers continue to study new treatments of aplastic anemia.