Abstract
A 3-month-old female infant with agammaglobulinemia developed hypergammaglobulinemia with M-protein. She contracted chronic active Epstein-Barr virus (EBV) infection with generalized lymph nodes swelling and hepatosplenomegaly. Serum IgG antibody to EBV viral capsid antigen was increased, whereas antibody to EBV-determined nuclear antigen was negative during her illness. Lymph node biopsy showed EBNA-containing cells. She died of malignant lymphoma 30 months after the onset of the disease. EBV-DNA was detected in enlarged lymph nodes at autopsy by southern blotting analysis. These data suggest that chronic EBV infection might have a causal relationship to the development of malignant lymphoma. Her elder sister and brother died of malignant lymphoma at the age of 20 months and of pneumonia at the age of 3 months, respectively. From these data, the clinical and laboratory findings in our patient may represent a new variant of hereditary immunodeficiency disease.
