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Immunodeficiency with hyperimmunoglobulin M is a rare disorder characterized by recurrent infections, low or absent IgG, IgE, and IgA levels, and normal or elevated levels of IgM and IgD. [ 2 ] : 84
Five different hypergammaglobulinemias are caused by an excess of immunoglobulin M (IgM), and some types are caused by a deficiency in the other major types of immunoglobulins. Paraproteinemia or monoclonal gammopathy is the presence of excessive amounts of a single monoclonal gammaglobulin (called a paraprotein ) in the blood.
Immunoglobulin M (IgM) is the largest of several isotypes of antibodies (also known as immunoglobulin) that are produced by vertebrates. IgM is the first antibody to appear in the response to initial exposure to an antigen ; [ 1 ] [ 2 ] causing it to also be called an acute phase antibody.
IgM nephropathy; Other names: immunoglobulin M nephropathy (IgMN) Antibody type IgM scheme. Blue - heavy chains, Yellow — light chains, Dark yellow/blue — variable regions (ends are binding sites), Light yellow/blue — constant regions, Green — disulfide bridges, Black — J chain
Hyper IgM syndrome is a rare primary immune deficiency disorders characterized by low or absent levels of serum IgG, IgA, IgE and normal or increased levels of serum IgM. [ 8 ] They are resulting from mutations in the pathway from B-cell activation to isotype class switching.
Most hypergammaglobulinemias are caused by an excess of immunoglobulin M (IgM), because this is the default immunoglobulin type prior to class switching. Some types of hypergammaglobulinemia are actually caused by a deficiency in the other major types of immunoglobulins, which are IgA, IgE and IgG.
Different genetic defects cause HIgM syndrome, the vast majority are inherited as an X-linked recessive genetic trait and most sufferers are male. [ 7 ] [ 1 ] [ 2 ] [ 3 ] [ 12 ] [ 4 ] IgM is the form of antibody that all B cells produce initially before they undergo class switching .
Immunoglobulin M (IgM) levels are reduced, IgA and IgE are elevated, and IgG levels can be normal, reduced, or elevated. [4] In addition to thrombocytopenia, WAS patients have abnormally small platelets (i.e. microthrombocytes) and ~30% also have elevated eosinophil counts (i.e. eosinophilia). [5]