Immunodeficiency with hyper-IgM (HIM) is a rare primary immunodeficiency characterized by the production of normal to increased amounts of IgM antibody of questionable quality and an inability to produce sufficient quantities of IgG and IgA.
What infections cause high IgM levels?
Viral and bacterial infections are the most common causes of high IgM levels. Some autoimmune disorders, including: Type 1 diabetes [35] Multiple sclerosis [36]
Is Hyper IgM Syndrome a primary immunodeficiency?
Hyper IgM syndromes are a group of rare disorders in which the immune system does not function properly. They are classified as rare primary immunodeficiency disorders, which are a group of disorders characterized by irregularities in the cell development and/or cell maturation process of the immune system.
What is immunodeficiency with hyper IgM?
Abstract. Immunodeficiency with hyper-IgM (HIM) is a rare disorder characterized by recurrent infections associated with low IgG and IgA, and normal to increased IgM serum levels. Both primary and secondary forms of HIM syndrome have been reported.
What causes IgM deficiency?
Other causes of decreased levels of serum IgM (i.e., secondary IgM deficiency) are episodes of infection, thymic hypoplasia, celiac disease, autoimmune disease, and certain adult malignancies; and other PIDs (Wiskott-Aldrich syndrome, DOCK8 deficiency, ataxia-telangiectasia, CVID, and XLA, in combination with IgG and …
What is immunodeficiency with hyper-IgM?
How common is CD40 ligand deficiency?
CD40 ligand deficiency is an uncommon primary immune deficiency with an estimated annual incidence of 1 in 106 (1).
How do you test for Hyper IgM?
Diagnosis of Hyper-IgM Syndrome Serum Ig levels are measured; normal or elevated serum IgM levels and low levels or absence of other immunoglobulins support the diagnosis. Flow cytometry testing of CD40 ligand expression on T-cell surfaces should be done. When possible, the diagnosis is confirmed by genetic testing.
