Abstract
A 7 month-old-boy with severe combined immunodeficiency suffered from cough, diarrhea, and intractable candidiasis, and died of a respiratory failure by giant cell pnemonia at 13 month-old. Immunological studies revealed low numbers of T cells, poor responses of his lymphocytes to mitogens, and markedly increased levels of surface immunoglobulin bearing cells. Serum concentrations of IgA and IgM were normal and IgG was high with M component, but functional antibody responses were absent. A plasma cell was absent in bone marrow and lymph nodes at autopsy. It was suggested that the maturation of B cell to plasma cell was impaired.
In addition, a tumor was found at his left upper arm on admission. The biopsy revealed that the tumor was a lymphoproliferative disease and consisted of the cells resembled mature lymphocytes. None of cells formed E rosette and had the surface immunoglobulin. The tumor cells infiltrated the muscles and fractured the left humerus. However, the tumor regressed spontaneously. At autopsy, the tumor was not found at the left upper arm but a solitary tumor with lymphoproliferation was found in the liver. It can not be denied that transfer factor and transplanted fetal thymus had an effect on the tumor regression. It is suggested that the tumor was not malignant but a monoclonal lymphocyte proliferation responsed to certain stimuli. Transplantations of the fetal thymus and the liver were unsuccesful in restoring immunity.
