Which medications in the drug class Immunoglobulins are used in the treatment of X-Linked (Bruton) Agammaglobulinemia?

Updated: Mar 26, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Immunoglobulins are the mainstay of therapy. Passively supply a broad spectrum of IgG antibodies against bacterial, viral, parasitic, and mycoplasmic antigens. Check IgG levels every 3 months and then every 6 months when stable. The goal is to maintain IgG trough levels greater than 500 mg/dL in serum. Check liver function and kidney function 3-4 times a year.

Immune globulin intravenous (Gamimune, Gammar-P, Sandoglobulin, Gammagard)

Immune globulin intravenous neutralizes circulating myelin antibodies through anti-idiotypic antibodies; down-regulates proinflammatory cytokines, including INF-gamma; blocks Fc receptors on macrophages; suppresses inducer T and B cells and augments suppressor T cells; blocks complement cascade; promotes remyelination; and may increase CSF IgG (10%).

Adjust the dose and interval according to individual needs.

Symptomatic adverse effects may be alleviated by premedicating with acetaminophen, diphenhydramine, or methylprednisolone (Solu-Medrol).

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