Roentgenologic Features of Lymphoid Hyperplasia of the Small Intestine Associated with Dysgammaglobulinemia

Abstract

In 1966 Hermans and his co-workers (1) reported eight cases of dysgammaglobulinemia associated with nodular lymphoid hyperplasia of the small intestine. Characteristically, these patients demonstrated an unusual susceptibility to infection, chronic or intermittent diarrhea, reduced concentration of gamma globulins, and roentgenologic evidence of nodular defects in the intestine. Since that report, two more patients have been observed with this combination of findings. The present report is primarily concerned with the roentgenologic appearance of these nodular defects in 10 patients. These tiny, nodular, filling defects are usually evident on films of the upper part of the small intestine and are located primarily in the duodenum and jejunum, although they may also be seen in the terminal part of the ileum and the right colon. Clinical Study All the 10 patients (6 men and 4 women) gave a history of either unusual susceptibility to infection or chronic diarrhea which had persisted for various periods. The unusual susceptibility to infection was usually manifested as pneumonia or upper respiratory-tract infection. When the patients were free of infection, the hemoglobin levels, erythrocyte counts, sedimentation rates, and leukocyte total and differential counts were essentially normal. Diarrhea, either chronic or intermittent, related to the onset of susceptibility to infection was the other major complaint. Mild degrees of steatorrhea were present in about half the patients. Stool examination for parasites and ova revealed the presence of Giardia lamblia in 6 patients. All patients had significant hypogammaglobulinemia. The total serum protein concentration was decreased in the majority of patients but was never less than 5.1 g/100 ml. Although excessive gastrointestinal loss of serum protein has been well established in a number of diseases, including Menetrier's disease (2), malignant diseases of the stomach (3), inflammatory disease of the small and large intestines (4), and nontropical sprue (5), this did not seem to be a factor in these patients. If hypogammaglobulinemia occurs as a consequence of gastrointestinal protein loss, it is almost always associated with hypoalbuminemia or is part of a generalized hypoproteinemia. Also, there is usually only mild hypogammaglobulinemia in excessive gastrointestinal protein loss associated with hypoalbuminemia. In these 10 patients, however, hypogammaglobulinemia existed in every patient, while hypoalbuminemia occurred only minimally in three. Significant immunoglobulin deficiency was determined by quantitative immunoelectrophoresis. The relationship of lymphoid hyperplasia to the incidence of G. lamblia in the stool is not known.