1 Departments of Paediatrics and Medicine, State University, Utrecht, The Netherlands
A report is presented of a girl who died at the age of 7 months, after having developed multiple infections. She was the fourth child in one family to die in this way. Mention is made of the importance of using immunologic techniques in serum examination in children with recurrent infections. By these techniques, a marked immune globulin deficiency was demonstrated in this case, and large quantities of paraproteins were demonstrated. It was impossible to diagnose any of the diseases known to be associated with paraproteinemia. A correlation is suggested between the paraproteinemia and the marked increase in plasma cells found at necropsy in the spleen, thymus and lymph glands. It is suggested that the case may have involved a hereditary inborn error of metabolism of the plasma cells. Therapeutic attempts with antibiotics and human gamma-globulin were unsuccessful. The fact that administration of gamma-globulin caused a marked increase in a paraprotein fraction indicates a disturbance in the protein metabolism.