Agranulocytosis are congenital and acquired. Acquired agranulocytosis are of two types - myelotoxic agranulocytosis (arising for example when exposed to ionizing radiation or cytotoxic drugs) and immune agranulocytosis, in which blood poyavlyayutsya autoantibodies (e.g., in diseases such as lupus erythematosus, autoimmune thyroiditis), or the antibodies to granulocytes appearing after taking drugs and purchasing when ingested after connecting to the properties of the protein antigen.
Proceeds agranulocytosis is typically acute, and mortality in this disease is 80%.
Eliminating causative factors
The very first action in the fight against agranulocytosis- Is the elimination of causal factors (stopping myelotoxic agents, eliminating the influence of myelotoxic chemicals, ionizing radiation, infection). Very often this leads to self-restoration of normal hematopoiesis.
Creating a sterile environment for patient
Cleanliness plays a role in the treatment ofdisease, so patients agranulocytosis should be placed in aseptic conditions (special boxes or chambers) should be provided kvartsevanie these chambers, limit visiting sick relatives. These events serve as prevention of infectious complications, which often take a severe course and may be the cause of death of patients agronulotsitozom.
Prevention and treatment of infectious complications
Prevention of infectious complications - is veryIt is important and it is the appointment mielonetoksichnyh antibiotics. Although agranulocytosis with the number of white blood cells to 1,5-109 / l, as a rule, antibiotic therapy is not appointed. Antibiotic treatment is performed before the release of agranulocytosis. During this treatment should also be used antifungals-mikostatiki (Nystatin, Levorinum et al.). In the treatment of infectious complications of agranulocytosis is also recommended to use intravenous immunoglobulin at a dose of 400 mg / kg single dose and intravenous antistaphylococcal plasma 100-150 ml of 1 times a day for 4-5 days.
Transfusion of leukocyte mass
With a pronounced decrease in white blood cells (whenantileykotsitarnyh absence of antibodies) Some hematologists recommend to do a transfusion of leukocyte mass or leukocytes thawed 2-3 times a week before the release of the state of agranulocytosis. However, it is necessary to prevent sensitization (sensitization) patients transfused leukocytes leukopenia and worsening try to pick up a lot of leukocyte to be compatible with a particular patient leukocytes.
Treatment with glucocorticoids
Glucocorticoid drugs are usedmainly in the immune agranulocytosis. Glucocorticoids stimulate and inhibit the production of granulocytopoiesis antileykotsitarnyh antibodies. In this case, prednisolone is usually used in a daily dose of 40 to 100 mg to normalize the number of leukocytes with subsequent tapering.
In the treatment of agranulocytosis oftenleykopoeza used stimulants, for example, sodium nukleinat 5 ml of 5% solution of 2 times per day administered intramuscularly or ingest 0.2-0.4 g 3-5 times a day; leucogen 0.02 g 3 times a day for pentoksil 0.1-0.15 g 3-4 times a day after meals. these drugs course of treatment is 2-4 weeks, depending on the severity of the disease.
Apply as colony stimulating factors - molgramostim, leykomaks of 3-10 mg / kg, administered subcutaneously for 7-10 days.
In severe intoxication (poisoning)organism detoxification therapy is performed: in this case gemodez intravenously administered 400 ml of 1 day, 5% glucose solution to 500 ml of isotonic sodium chloride solution or Ringer 0.5-1 liter.