Indications for use

1) Replacement therapy for primary immunodeficiency (PID) with impaired antibody production:
congenital agammaglobulinemia and hypogammaglobulinemia;
General variable immune insufficiency;
severe combined immune insufficiency;
Wiskott-Aldrich syndrome.
2) Replacement therapy for secondary immunodeficiency:
multiple myeloma with severe secondary hypogammaglobulinemia and recurrent bacterial infections when pneumococcal vaccination is ineffective;
chronic lymphoid leukemia with a severe form of secondary hypogammaglobulinemia and recurrent bacterial infections when preventive antibacterial therapy is ineffective;
congenital acquired human immunodeficiency syndrome (AIDS) in children with recurrent infections;
hypogammaglobulinemia in patients after allogeneic hematopoietic stem cell transplantation;
severe forms of bacterial and viral infections; postoperative complications accompanied by bacteremia; generalized infections (sepsis).
3) as an immunomodulatory agent:
in idiopathic thrombocytopenic purpura (ITP) in children or adults with a high risk of bleeding or before surgery to correct the number of platelets;
in Guillain-Barré syndrome;
in Kawasaki disease;
in other autoimmune diseases (systemic lupus erythematosus, vasculitis);
for urticaria.

DOSING MODE

Before injection, the drug is warmed to room temperature or body temperature.
The drug should be administered only as intravenous infusions, initially at a rate of 0.5 mg/kg body weight (15-20 drops per minute or 0.75-1 ml / min)
in the first 10-15 minutes, then-at the rate of 30-40 drops per minute (1.5-2 ml/min). If well tolerated the infusion rate can be gradually increased
up to 8 mg / kg body weight / min (approximately 10 ml / min). In patients with primary immunodeficiency who have well tolerated substitution therapy with the drug, the rate of infusion can be gradually increased to the maximum value
12 mg / kg body weight/min.
The dose and dosage regimen depend on the indication for use. In the case of substitution therapy, the dose of the drug can be selected individually for each patient, depending on the pharmacokinetic parameters and clinical response. The following doses are recommended as a guide

Contraindications

Contraindications for the use of the drug are:
hypersensitivity to the active substance or any other component of the drug;
hypersensitivity to homologous immunoglobulins, especially in very rare cases of immunoglobulin A (IgA) deficiency, when the patient has IgA antibodies.
In cases of severe sepsis, the only contraindication for use is anaphylactic shock to human blood products in the anamnesis.
Side effect
Adverse reactions according to numerous clinical studies on intravenous immunoglobulin preparations, presented below, are listed according to the damage to organs and organ systems and frequency of occurrence. The frequency of occurrence is defined as follows: very often (>1/10), often (>1/100 and <1/10), infrequently (>1/1000 and <1/100), rarely (>1/10 000 and <1/1000), very rarely (<1/10 000, including individual cases).
Immune system disorders: very rare-anaphylactic reactions (including anaphylactic shock), angioedema, facial edema.
Disorders of the blood and lymphatic system: infrequently-anemia, anisocytosis, leukopenia, hemolysis.
Disorders of the nervous system: very often-headache; infrequently-dizziness, discomfort in the head, drowsiness; rarely-aseptic meningitis.
Disorders of the heart: rare – palpitations (palpitation).
Vascular disorders: often-arterial hypertension; infrequently-arterial hypotension, hot flushes, peripheral vascular disorders; very rarely-thromboembolic complications.
Disorders of the respiratory system, chest and mediastinal organs: infrequently-difficulty breathing, a feeling of tightness in the throat
Disorders of the gastrointestinal tract: often-nausea; infrequently-diarrhea, pain in the epigastrium.
Disorders of the liver and biliary tract: infrequently-hyperbilirubinemia.
Skin and subcutaneous tissue disorders: often-urticaria, rashes; infrequently-itching, night sweats.
Disorders of the musculoskeletal and connective tissue: often-back pain; infrequently-neck pain, pain in the extremities.
Disorders of the kidneys and urinary tract: infrequently-proteinuria.
General disorders and disorders at the injection site: often-chills, fatigue, fever, asthenia, flu – like condition; infrequently-chest pain, General malaise, fever, pain at the injection site.
Laboratory and instrumental data: rarely is the increase in the concentration of bound and unbound bilirubin in the blood, positive direct Coombs test, positive indirect Coombs test, increased activity of lactate dehydrogenase in the blood, decrease in hematocrit, increase of aspartate aminotransferase activity, an increased concentration of blood creatinine, decreased blood pressure, increased blood pressure, increased body temperature, decreased hemoglobin.