THYMIC ALYMPHOPLASIA WITH HYPER-IgE-GLOBULINEMIA

Abstract

The clinical and autopsy findings of a 5-month-old patient with thymic alymphoplasia and increased serum IgE levels are reported. A congenital heart lesion was diagnosed at 15 days of age, eczema at 1 month of age, and infections of the skin, ears, and respiratory tract recurred until death. Peripheral lymphocytopenia and eosinophilia, and increased concentrations of serum IgE (16.2µg/ml) and IgM were present. The serum contained normal levels of IgG and IgA. Isohemagglutinins and viral neutralizing antibodies were not present in the serum. Bacterial and drug antibodies were present, as demonstrated by PK reaction. At autopsy the thymus was hypoplastic and without Hassall's corpuscles. There were few small lymphocytes but follicular lymphocytes and plasma cells were relatively normal in lymph nodes. IgE forming cells were found in the lymph nodes and lamina propria of intestine by fluorescent antibody technique. Peripheral lymphocytes showed blastlike transformation but not mitosis. The findings suggest that the thymus plays a role in the regulation of IgE synthesis.