How is hypogammaglobulinemia in primary immunodeficiencies treated?

Updated: Dec 26, 2018
  • Author: Elizabeth A Secord, MD; Chief Editor: Michael A Kaliner, MD  more...
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Answer

In these disorders, B-cell function and or numbers can be impaired, leading to an inability to generate effective antibody responses. The disorders for which immunoglobulin is variably used fall into three categories: (1) Milder forms of combined immunodeficiencies (such as that caused by partially functional mutations in recombinase-activating genes [RAG]) and (2) Combined immunodeficiencies with associated or syndromic features (such as Wiskott-Aldrich syndrome).

Diseases of immunodysregulation (such as CD27 deficiency). In SCID, immunoglobulin replacement is also necessary in the post-transplantation period, during gene therapy or enzyme replacement (for adenosine deaminase deficiency), until B-cell function is restored.


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