SUMMARY A patient with nodular lymphoid hyperplasia, who manifested defects in cellular immunity, is presented. The clinical course is discussed and a review included of the 23 patients thus far reported with nodular lymphoid hyperplasia and hypogammaglobulinaemia.Acquired idiopathic hypogammaglobulinaemia can be associated with a sprue-like syndrome in about 20% of patients (Gitlin, Gross, and Janeway, 1959). Hermans, Huizenga, Hoffman, Brown, and Markowitz (1966) described the association of hypogammaglobulinaemia with nodular lymphoid hyperplasia of the small intestine, recurrent upper respiratory infections, the malabsorption syndrome with steatorrhoea, and infestation with Giardi lamblia. Nodular lymphoid hyperplasia is relatively rare and is not found in the vast majority of patients with acquired idiopathic hypogammaglobulinaemia. Our purpose is to report a patient with this disorder and to review the literature relating to this subject.
Case HistoryThe patient, a male aged 62, presented in November 1969 complaining of loss of appetite and vomiting for two weeks and weight loss of 20 kg over one month. His previous health had been good apart from the symptoms of chronic bronchitis for 30 years. Examination showed evident weight loss and pallor. Haemoglobin was 9.5 g per 100 ml with macrocytosis of the peripheral blood film. Megaloblasts were present in the sternal marrow biopsy. An augmented histamine test revealed achlorhydria. After a standard Schilling test no radioactive vitamin B12 was excreted in the urine and the addition of intrinsic factor improved the 24-hour urinary excretion to only 7 % of the administered dose (normal > 12 %). Total serum protein was 4.6 g per 100 ml (albumin 2-9 g, total globulin 1-7 g). A barium meal showed an atrophic mucosal pattern and poor peristalsis suggestive of linitis plastica.After transfusion with 4 pints of blood a laparotomy revealed no abnormality except for a few enlarged mesenteric nodes, one of which was