Granulocytes are a population of leukocytes containing core. In turn, they are divided into three subpopulations - basophils, neutrophils and eosinophils. The name of each of them is due to the peculiarities of staining that indicate the biochemical composition.
Conventionally, it is believed that neutrophils provide antibacterial immunity, eosinophils - an antiparasitic. Thus, the main feature and the most significant threat to the life of the patient factor is the sudden loss of the ability to resist various infections.
The causes of agranulocytosis
In autoimmune syndrome form in the functioning of the immune system certain failure occurs, resulting in which it produces antibodies (so-called autoantibodies), granulocytes attackers, thereby causing them death.
Common causes of disease:
- Viral infections (caused by the Epstein - Barr virus, cytomegalovirus, yellow fever, viral hepatitis) are usually accompanied by mild neutropenia, however, may develop in some cases, agranulocytosis.
- Ionizing radiation, radiotherapy, chemicals (benzene), insecticides.
- Autoimmune diseases (e.g. lupus erythematosus, Hashimoto's thyroiditis).
- Severe generalized infections (both bacterial and viral).
- Emaciation.
- Genetic disorders.
Medicaments can cause agranulocytosis caused by direct suppression of hemopoiesis (cytostatics, valproic acid, carbamazepine, beta-lactam antibiotics), or acting as haptens (preparations of gold, and antithyroid drugs etc.).
shape
Agranulocytosis is congenital or acquired. Congenital is associated with genetic factors and is extremely rare.
Acquired forms of agranulocytosis revealed a frequency of 1 case per 1,300 people. It described above, that, depending on the characteristics of the pathological mechanism underlying death of granulocytes, are the following kinds of it:
- myelotoxic (cytotoxic disease);
- autoimmune;
- hapten (drug).
Also known genuinnaya (idiopathic) form, in which the cause of development can not be established agranulocytosis.
By the nature of the flow agranulocytosis is acute or chronic.
symptoms of agranulocytosis
Symptomatology begins to arise after antileykotsitarnyh content of antibodies in the blood reaches a certain limit. In this regard, the appearance of agranulocytosis in the first place a person concerned about such symptoms:
- poor general health - severe weakness, sweating and pallor;
- elevated temperature (39º-40º), chills;
- appearance of ulcers in the mouth, tonsils and soft palate. In this case, the person feels pain in the throat, it is difficult to swallow, there drooling;
- pneumonia;
- sepsis;
- ulcerative lesions of the small intestine. The patient feels bloating, it appears loose stools, cramping abdominal pain.
Furthermore agranulocytosis common manifestations are changes in blood analysis:
- in humans is drastically reduced total number of leucocytes;
- drop occurs neutrophil level, until the complete absence;
- relative lymphocytosis;
- increased erythrocyte sedimentation rate.
To confirm the presence of agranulocytosis in humans administered bone marrow examination. Once diagnosed, the next stage - treatment of agranulocytosis.
diagnosis of agranulocytosis
Group of potential risk for the development of agranulocytosis consists of patients with severe infectious disease, receiving radiation, cytotoxic or other drug therapy suffering connective tissue. Data from clinical diagnostic value is a combination of hyperthermia, ulcerative necrotic lesions visible mucous and hemorrhagic manifestations.
Most important for the confirmation of agranulocytosis is to study the general analysis of blood and bone marrow puncture. Painting characterized peripheral blood leukopenia (1-2h109 / l), granulocytopenia (less 0,75h109 / l) or agranulocytosis, mild anemia in severe degrees - thrombocytopenia. When myelograms examination revealed reduction in the amount myelokaryocytes, reducing the number and the violation neutrophil maturation of the germ cells, the presence of a large number of plasma cells and megakaryocytes. a determination of anti-neutrophil antibodies to confirm the autoimmune nature of agranulocytosis.
All patients with agranulocytosis shown radiography lungs, repeated studies blood on sterility, the study of the biochemical analysis of blood, dental consultation and otolaryngologist. Agranulocytosis necessary to differentiate from acute leukemia, aplastic anemia. It is also necessary exclusion of HIV status.
complications
Mielotoksicskie disease may have the following complications:
- Pneumonia.
- Septicemia (blood poisoning). Often there staphylococcal sepsis type. The most dangerous complication for the patient's life;
- Perforation of the intestine. The ileum is most sensitive to the formation of through-holes;
- Severe the swelling of the mucous intestine. In this case the patient has a bowel obstruction;
- Acute hepatitis. Often, in the treatment of epithelial formed hepatitis;
- Education necrosis. It refers to infectious complications;
- Septicemia. The more the patient is sick myelotoxic kind of disease, the harder it is to eliminate its symptoms.
If the disease is caused by hapten or arises from the weakness of the immune system, the symptoms of the disease manifests itself most clearly. Among the sources that cause the infection are isolated saprophytic flora, which include Pseudomonas or E. coli. In this case, the patient is severe intoxication, the temperature rise to 40-41 degrees.
Agranulocytosis how to treat?
In each case, the origin is considered agranulocytosis, degree of severity, presence of complications, the general condition of the patient (sex, age, concomitant diseases, etc.).
Upon detection of agranulocytosis shows a comprehensive treatment that includes a variety of activities:
- Hospitalization in the hematology department of a hospital.
- Putting patients in a box room, where he regularly held air disinfection. Completely sterile conditions to help prevent infecting a bacterial or viral infection.
- Parenteral nutrition is indicated in patients with necrotizing enteropathy.
- A thorough oral hygiene is of frequent rinses with antiseptics.
- Causal treatment is aimed at eliminating the causative agent - the cessation of radiation therapy and administration of cytotoxic drugs.
- Antibiotic therapy given to patients with purulent infection and severe complications. To do this, use just two of a wide spectrum of the drug - "neomycin", "Polymyxin", "oletetrin". Treatment complementary antifungal agents - "Nystatin" "Fluconazole", "Ketoconazole".
- Leukocyte concentrate transfusion, bone marrow transplantation.
- The use of glucocorticoids in high doses - "prednisolone", "Dexamethasone", "Diprospan".
- Stimulation leykopoeza - "leucogen", "Pentoxyl", "Leykomaks".
- Detoxification - parenteral administration "gemodez" glucose solution, isotonic sodium chloride solution, a solution "Ringers".
- Correction of anemia - eg. IDA for iron supplementation, "Sorbifer durules", "Ferrum lek".
- Treatment of hemorrhagic syndrome - platelet transfusions, administering "Dicynonum", "aminocaproic acid", "menadione".
- oral solution Processing "levorin" sea buckthorn oil lubrication ulcers.
Forecast treatment of the disease, is generally favorable. Its appearance may worsen tissue necrosis and infectious ulcers.
preventive measures
Prevention agranulocytosis, mainly consists in carrying out a careful hematological monitoring during the course myelotoxic treatment preparations exclusion repeated medication previously caused the patient immune phenomena agranulocytosis.
Poor prognosis observed in the development of severe septic complications, recurrence of agranulocytosis hapten
