Immunoblastic lymphoma of small intestine complicating late-onset immunodeficiency

Abstract

A malignant lymphoma of the small bowel developed in a 75-year-old woman who had severe late-onset hypogammaglobulinemia with intestinal malabsorption and nodular lymphoid hyperplasia. The tumor arose in the midst of the abnormal lymphoid follicles and appeared to be of B-immunoblastic type. Immunoperoxidase staining, however, failed to reveal immunoglobulins in the tumor cells. In vitro immunologic studies showed that immunoglobulin production by the patient's peripheral lymphocytes had decreased and that this decrease was mediated by hydrocortisone-sensitive suppressor cells. These findings suggest that nodular lymphoid hyperplasia of the intestine in the context of severe immunodeficiency is a premalignant condition.