A Familial Case of Hyper-IgM Immunodeficiency

Abstract

A 22-year-old male was diagnosed as having immunodeficiency with hyper-IgM based upon recurrent pneumonia, marked elevation of serum IgM and markedly decreased level of IgG. IgG- or IgA-bearing B cells were not detected in peripheral blood while a number and a proportion of peripheral blood T lymphocytes were normal. Peripheral blood lymphocytes from this patient proliferated normally in response to T-independent and T-dependent B cell mito-gens, and to T cell mitogens. Furthermore, the same type of dysgammaglobulinemia with increased IgM was found in the patient’s father and brother. From these observations, it is suggested that it is a rare case of autosomal dominant or polygenal inheritance of hyper-IgM immunodeficiency.

An Adult Diagnosed as Hyper-IgM Immunodeficiency Syndrome.

“…34, No. 7 (July 1995) patterns such as autosomal dominant or recessive, and acquired forms have also been reported (5,(9)(10)(11)(12)(13)(14)(15). Acquired diseases are associated with congenital or late-onset rubella syndrome, chronic lung disease, hemolytic anemia, or tumors, and the use of anti-epileptic drugs such as diphenylhydantoin (5).…”