Abstract
A 2-month-old boy was admitted on August 3, 1973, because of high fever, hepatosplenomegaly and leukocytosis. After admission, he continued to develop high fever, heomrrhagic diathesis including melena and petechial hemorrhages scattered over the entire body and eczematous skin lesions with the concomitant development of suppuration. Cultures of blood, urine and secretions from suppurative skin lesions grew Enterococcus, Klebsiella and Staphylococcus aureus, respectively, at the different stages of the whole course of the disease.
Among other laboratory findings, anemia, leukocytosis with striking leukoerythroblastosis, thrombocytopenia, hyperimmunoglobulinemia G, A, M and E and significant reduction of isohemagglutinin titer were noteworthy. The vigorous antimicrobial therapy was instituted immediately after admission and was continued throughout the hospital course, but gave no remarkable improvement. Consequently his general condition was deteriorated gradually and he died on October 29, 1973.
An autopsy performed about 3 hours after the death revealed severe bronchopneumonia, a number of cytomegalic inclusion bodies in submaxillary glands and kidneys, and markedly atrophic thymus gland weighed 0.5gm.
Moreover, the histology of the thymus gland showed indistinct “corticomedullary” boundary attributable to lymphocyte depletion in the thymic cortex. Several lymph nodes revealed profound lymphocyte depletion especially in thymus-dependent paracortical area with no definite development of reticular hyperplasia and plasmacytosis.
Beside these findings, on family survey, 3 other boys in maternal side were found to have developed symptoms and signs similar to those in this case and have succumbed to overwhelming infections within a year after birth.
Based on the clinical, laboratory and histological findings above described, we discussed hyperimmunoglobulinemia E and cytomegalic inclusion disease in this case chiefly in relation to the striking atrophy of the thymus gland.
It seems likely that marked elevation of serum IgE and other classes of Ig in this case was ascribed to T cell deficiency secondary to thymic atrophy, and cytomegalovius might be transferred into the patient via multiple fresh blood transfusions.
