Hyper-IgM Immunodeficiency Syndrome

**Definition:** A rare inherited immunodeficiency syndrome characterized by normal or elevated serum IMMUNOGLOBULIN M levels with absence of IMMUNOGLOBULIN G; IMMUNOGLOBULIN A; and IMMUNOGLOBULIN E. It results in a profound susceptibility to BACTERIAL INFECTIONS and an increased susceptibility to OPPORTUNISTIC INFECTIONS. Several subtypes of hyper-IgM immunodeficiency syndrome exist depending upon the location of genetic mutation.

**Tree numbers:** - C15.378.147.333.249 - C16.320.413 - C16.320.798.625 - C20.673.430.250 - C20.673.795.625

**Synonyms:** - Hyper-IgM Syndrome - Immunodeficiency with Hyper-IgM Syndrome