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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.
Different genetic defects cause HIgM syndrome, the vast majority are inherited as an X-linked recessive genetic trait and most with the condition 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) 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.
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
Different genetic defects cause HIgM syndrome, the vast majority are inherited as an X-linked recessive genetic trait and most with the condition are male. [ 7 ] [ 2 ] [ 3 ] [ 4 ] [ 12 ] [ 5 ] IgM is the form of antibody that all B cells produce initially before they undergo class switching .
Both are characterized by an immune system malfunction which may cause similar symptoms, such as rash, swelling, or fatigue, but the cardinal cause or mechanism of the diseases are different. A key difference is a malfunction of the innate immune system in autoinflammatory diseases, whereas in autoimmune diseases there is a malfunction of the ...
The work-up for diagnosis is complex and the condition can be over-diagnosed. [1] [2] [better source needed] People diagnosed with warm autoimmune hemolytic anemia (WAIHA) caused by immunoglobulin G (IgG) [3] may also have a high number of immunoglobulin M antibodies. These antibodies are active at room temperature, but are believed to be ...
IgM nephropathy or immunoglobulin M nephropathy (IgMN) is a kind of idiopathic glomerulonephritis that is marked by IgM diffuse deposits in the glomerular mesangium. [1] IgM nephropathy was initially documented in 1978 by two separate teams of researchers.