Clinical Uses
IVIG is administered either to protect the recipient from infection or to suppress immune-mediated or inflammatory disease processes. While it is used off-label in nearly 100 conditions with probable or proven benefit, it is FDA approved for only eight:
- Immune thrombocytopenic purpura (ITP): generally chronic, treatment-refractory ITP or given to emergently increase a platelet count in a bleeding ITP patient.
- Primary immunodeficiency.
- Secondary immunodeficiency.
- Pediatric HIV infection.
- Kawasaki disease.
- Prevention of GVHD in bone marrow transplant patients.
- Incompatible solid organ transplants.
- Chronic inflammatory demyelinating polyneuropathy (CIDP).
- Common off-label uses include:
- Hematologic: autoimmune hemolytic anemia, heparin-induced thrombocytopenia, autoimmune neutropenia, post-transfusion purpura, aplastic anemia, hypogammaglobulinemia associated with multiple myeloma.
- Renal/vasculitis: membranous nephropathy, IgA nephropathy, lupus nephritis, GPA.
- Neuromuscular: polymyositis, dermatomyositis, Guillain-Barré syndrome, myasthenia gravis.
- Dermatologic: pemphigus vulgaris, toxic epidermal necrolysis and Stevens-Johnson syndrome.
Mechanisms of Action
Incompletely understood, but likely include the following:
- Regulation and inhibition B-cells and T-cells.
- Suppression of antibody production.
- Modulation of complement.
- Reduction of apoptosis.
- Inhibition of cell-mediated cytotoxicity.
- Inhibition of leukocyte adhesion.
- Blockage of macrophage receptors.
Risks
- During administration:
- Flushing, tightness, back pain, nausea, chills, diaphoresis, headache, fever, hypotension.
- Anaphylaxis (rare reaction in patients with high titers of anti-IgA antibodies).
- Dose-related adverse effects:
- Vascular: cerebral and coronary thromboses.
- Infection: as with any blood product, small risk of viral hepatitis or HIV.
- Neurologic: acute aseptic meningitis.
- Hematologic: hemolysis (self-limited) and neutropenia (transient).
- Renal: reversible renal impairment (can cause acute renal failure from osmotic nephropathy).
- Electrolyte abnormalities: hyponatremia, pseudohyponatremia, decreased anion gap, false positive serologies.
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Karafin MS, Shaz BH. Chapter 37: Human Immunoglobulin Preparations In: Shaz BH, Hillyer DC, Abrams SC, Roshal M eds. Transfusion Medicine and Hemostasis: Clinical and Laboratory Aspects, 2e. San Diego, CA: Elsevier; 2013.
Mouthon L, Lortholary O. Intravenous immunoglobulins in infectious diseases: where do we stand? Clin Microbiol Infect 2003;9:333-338.
Sacher RA; Intravenous immunoglobulin consensus statement. J Allergy Clin Immunol 2001;108:S139-146.