Immunodeficiency with Hyper-lgM (Dysgammaglobulinemia Type I) Accompanied by Aortic Calcification

Abstract

Immunodeficiency with hyper Ig-M (dysgammaglobulinemia type 1) is characterized immunologically by decreased or absent serum levels of IgG, IgA, but elevated or normal IgM. First described by Rosen et al. in 1961, the syndrome is usually seen in young boys with recurrent bacterial infection, and autoimmune diseases are the frequent manifestation. We present a 4 9/12 year old boy who showed imnunologic findings compatible with immunodeficiency with hyper-IgM and associated with huge dilatation and calcification of the aorta. We studied the Band T cell function in vitro which suggested the excessive suppressor function of the patient's T lymphocytes, although special staining of the lymph node did not show predominant suppressor population.