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The first manifestations of any agranulocytosis are fever, stomatitis, tonsillitis. With myelotoxic syndrome, there is usually a moderately pronounced hemorrhagic syndrome (bruising and bleeding of the gums, nosebleeds): severe hemorrhagic syndrome is an infrequent complication of agranulocytosis. The defeat of the mucous membranes (necrosis and thrush) of the oral cavity and gastrointestinal tract is the most constant sign of agranulocytosis.
With myelotoxic agranulocytosis, it is due to the fact that, on the one hand, the disappearance of granulocytes makes possible microbial invasion, on the other hand, suppression of mitosis of epithelial cells of the mucous membrane by cytostatic factors violates its integrity.
The prognosis in most cases of melotoxic agranulocytosis under conditions of asepsis and timely powerful antibiotic therapy is favorable. The prognosis is sharply worsened by necrotizing enteropathy, as well as severe septic complications. The prognosis for immune agranulocytosis of drug origin in the conditions of baricitinib withdrawal of the hapten drug, steroid hormone therapy and in compliance with all the rules for managing such patients is in most cases favorable.
Treatment of agranulocytosis of any origin requires immediate elimination of the cause that caused it: a cytostatic drug, ionizing radiation, a hapten drug that provoked an immune conflict, and so on. Therapy of agranulocytosis complicating the course of rheumatoid arthritis or disseminated lupus erythematosus, first of all, should be directed against the underlying disease.
Steroid hormones are not indicated in myelotoxic agranulocytosis. With a decrease in the number of leukocytes to 50-200 cells in 1 μl, replacement therapy is necessary - the transfusion of leukocyte concentrate. More than 15-20 billion cells are transfused at once. In cancer patients, a good effect was noted with the use of leukocyte mass obtained from patients with chronic myeloid leukemia in the advanced stage of the disease. Bone marrow transplantation is used only for acute total irradiation in doses exceeding 600 rad per bone marrow.
Along with the use of pathogenetic agents, symptomatic therapy is also used: in the case of persistent hyperthermia up to 39-40 ° with myelotoxic agranulocytosis (but not immune!), Patients are given analgin up to 2-3 g per day or acetylsalicylic acid - 2 g per day.
In the treatment of immune agranulocytosis, a decisive role belongs to prednisolone at a dose of 1-1.5 mg per 1 kg of body weight or its analogs in adequate doses, and in the absence of an effect - 2-3 times more. In case of severe damage to the mucous membranes, the drug is administered parenterally. The course is 7-10 days or until the elimination of agranulocytosis. Replacement therapy with leukocyte mass transfusions is indicated only for infectious complications.
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To prevent infection, patients must be placed in boxes or isolators where aseptic conditions are created. Prevention of infectious complications with antibiotics is necessary when the number of granulocytes drops to 750 per 1 μl. Inside appoint a non-absorbable antibiotic neomycin up to 2-3 g per day (against E. coli), polymyxin B, 150-200 mg per day (against Pseudomonas aeruginosa). In addition, a broad-spectrum antibiotic is prescribed - garamycin 40-80 mg intramuscularly 2-3 times a day, or oxacillin 4-6 g per day by mouth, or olettrin up to 2 g per day by mouth. Be sure to take nystatin up to 10,000,000 - 15,000,000 U per day. For systematic mouth rinsing, a solution of levorin 1: 500 is used.