Hyper IgM Syndrome - An Immunodeficiency Disorder

A seven-year male child presented with history of recurrent life threatening infections associated with low IgA and IgG levels and significant elevation of IgM levels. He was diagnosed to have Hyper IgM syndrome. There was significant improvement after the immunoglobulin therapy. The child is now on regular immunoglobulin therapy and is free from recurrent infections. An index of suspicion to consider a possible diagnosis of immunodeficiency is stressed.