In their correspondence, Hennig et al propose to abandon the term "hyper-IgM (HIGM) syndrome" that has been widely used in the medical literature because of low sensitivity and specificity of IgM serum levels in defining the disease. Indeed, only a proportion of patients with the HIGM syndrome have elevated IgM levels at the time of diagnosis, and even fewer present with high IgM levels early in childhood, when correct recognition of the disease and prompt treatment would be most needed. Furthermore, elevated serum IgM levels are not unique to the HIGM syndrome but can also be found in other conditions such as ataxia-telangiectasia.
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