agranulocytosis
Agranulocytosis - a decrease in the number of leukocytes (less than 1000 in 1 mm) or the number of granulocytes (less than 1 in 750 ml of blood).. Agranulocytosis usually is a syndrome of a common disease. More common myelotoxic agranulocytosis (see. Cytostatic. Disease) and immune. The latter may be due to the appearance of autoantibodies (eg, systemic lupus erythematosus), and antibodies to granulocytes after taking the medication caught haptens (by ingestion of these medicines, combining with a protein antigen acquire properties). Hapten agranulocytosis develops under the influence diakarba (diamoksa) amidopirina, antipyrine, aspirin, barbiturates, isoniazid (tubazid) meprotan (meprobamate), phenacetin, butadiona, procainamide (procainamide), indomethacin, levamisole, sulfonamides, methicillin, trimethoprim (included in composition baktrima) hingamina (chloroquine) insecticides, clozapine (leponeks) and others.
The pathogenesis has not been studied. In autoimmune forms defeat the premature death of bone marrow granulocytes and their precursors due autoanti bodies. The mechanism of individual reactions to the introduction of the drug at a hapten agranulocytosis unclear. Once appeared, hapten agranulocytosis is invariably repeated when administered to the same drug-hapten.
The clinical picture of the disease is due to agranulocytosis. which is characterized by septic complications.. sore throat, pneumonia, etc. When hapten agranulocytosis granulocytes in the blood is usually not, but the number of lymphocytes, platelets, reticulocytes normal. Hemorrhage does not happen. In autoimmune agranulocytosis occasionally may cause antibodies to platelets and, if there is thrombocytopenic purpura, hemorrhagic. The clinical picture is reminiscent of cytostatic disease manifestations.
Treatment. Patients urgently hospitalized and placed under aseptic conditions (insulators, ultraviolet radiation from the air defense of the patient, the staff comes in masks, caps, shoe covers). Treatment of septic complications is similar to treatment for acute radiation sickness. Autoimmune shows agranulocytosis glucocorticoid in high doses (60-100 mg / day) to normalize the number of granulocytes in the blood, followed by gradual withdrawal of hormones. When hapten afanulotsitoze glucocorticoids are not effective. Treatment with immune agranulocytosis ballroom preferably carried out in a specialized hospital.
Forecast autoimmune agranulocytosis is determined by the underlying disease (systemic lupus erythematosus, rheumatoid arthritis, and so on. N.). Hapten agranulocytosis gives a high percentage (up to 80) deaths. Very compounded forecast haptenated agranulocytosis with repeated use of the drug has caused agranulocytosis against the background of the disease. As is often the doctor can not determine what kind of medication proved to hapten, it is necessary to exclude the use of patients not only during illness, but in the future (for life!) All suspected drugs applied directly to the development of agranulocytosis. This is usually is the prevention of recurrent agranulocytosis haptenated type.
- Diseases of the blood system
- Anemia (anemia)
- Gaucher's disease
- Vnekostnomozgovye hemoblastoses - gematosarkomy and lymphoma (limfotsitomy)
- Hemorrhagic diathesis and syndromes
- leukemoid reaction
- leukemias
- megakaryoblastoma
- Radiation sickness
- neutropenia hereditary
- paraproteinemic hemoblastoses
- thrombophilia hematogenous
- cytostatic disease
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