HYPERlgM Syndrome

Ramsay L Fuleihan, Section of Immunology, Department of Pediatrics, Yale University School of Medicine, New Haven, Connecticut, USA

The hyper-IgM syndrome is a rare immune deficiency disorder which was first described in 1961 in two independent reports by Rosen and colleagues and by Burtin. It is characterized by a defect in immunoglobulin isotype switching resulting in low or undetectable serum levels of IgG, IgA and IgE but normal or elevated IgM levels. Most patients exhibit an X-linked mode of inheritance; some families with affected females suggest autosomal recessive or dominant modes of inheritance. The hyper-IgM phenotype has also been associated with ataxia telangiectasia and can present as an acquired disorder that is associated with congenital rubella, administration of anti-epileptic drugs, chronic lung disease, autoimmune hemolytic anemia or tumors. In 1993, five different groups discovered that the X-linked hyper-IgM syndrome results from defects in the gene encoding CD40 ligand (CD40L), a glycoprotein expressed on activated T cells that drives immunoglobulin iso type switching in B cells through interaction with CD40.

How To Deal With Rosacea and Eczema

How To Deal With Rosacea and Eczema

Rosacea and Eczema are two skin conditions that are fairly commonly found throughout the world. Each of them is characterized by different features, and can be both discomfiting as well as result in undesirable appearance features. In a nutshell, theyre problems that many would want to deal with.

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