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Hyper IgM syndrome is a family of genetic disorders in which the level of Immunoglobulin M (IgM) antibodies is relatively high. The disorder causes immunodeficiencies, including a higher than normal susceptibility to various types of infections. Individuals with hyper-IgM syndrome typically also have a low number of infection-fighting neutrophil cells.
IgM is the form of antibody that all B cells produce initially, before they undergo class switching due to exposure to a recognized antigen. Healthy B cells efficiently switch to other types of antibodies as needed to attack invading bacteria, viruses, and other pathogens. Generally, in people with hyper IgM syndromes, the B cells keep making IgM antibodies because they can’t switch to a different kind of antibody. This results in an overproduction of IgM antibodies and an underproduction of all other types. Some hyper-IgM syndromes are actually deficiencies of the other immunoglobulin classes: IgA, IgG, and IgE.
Five types have been characterized:
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