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Hyper IgM Syndromes (Pediatric)

What are hyper IgM syndromes?

Hyper IgM syndromes comprise a group of primary immune deficiency disorders that are associated with elevated immunoglobulin M (IgM) and too little of other immunoglobulins such as IgG and IgA. People with hyper IgM syndromes may develop recurrent infections, severe infections, and, in some cases, opportunistic infections. Some forms have defects in their T cells, in addition to an antibody deficiency. Higher rates of cancer have been reported.

What is the treatment for hyper IgM syndromes?

Treatment may involve immunoglobulin replacement and antibiotics to protect against the opportunistic infection pneumocystis carinii pneumonia.

Some patients may benefit from granulocyte colony stimulating factor therapy. In some cases, bone marrow or cord blood stem cell transplantation is needed.