Nodular lymphoid hyperplasia with hypogammaglobulinaemia

Abstract

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

Nodular lymphoid hyperplasia of the bowel in primary hypogammaglobulinaemia: study of in vivo and in vitro lymphocyte function.

“…This is probably an underestimate, as barium follow-through examination may not reveal small nodules. Only two patients had giardiasis (Table 3) which is at variance with the findings of Ajdukiewicz (1972) who, in reviewing the literature, found that 15 of 21 patients with NLH had giardiasis. However, many of these cases were from North America where giardiasis accounts for most of the malabsorption in patients with hypogammaglobulinaemia (Ament et al, 1973).…”

“…The finding of a fibrous capsule around a nodule from MH is not consistent with either a germinal centre or Peyer's patch anology and was probably a 'chance' finding, as in all the other nodules examined there was no sign of such a capsule. Ajdukiewicz et al (1972) in their review, found no evidence that patients with NLH were identifiable by certain in vitro and in vivo immunological tests. Our attempts, using a wider variety of tests, confirm this view (Table 3).…”

Nodular lymphoid hyperplasia of the small intestine with Burkitt's lymphoma and dysgammaglobulinemia

“…The second case [1], however, did have associated dysgammaglobulinemia. The most common immunoglobulin abnormality in patients with NLH is absent or diminished IgA, but there is usually concomitant decreased IgG and absent or near absent IgM [5]. Solitary IgA deficiency, as in our patient, usually occurs in apparently healthy individuals [6].…”

Giardiasis: Nutritional Implications

“…Among a large number of patients with immunodeficiency syndromes, 80070 of those with intestinal manifestations had concurrent giardiasis [86]. Extraordinarily high incidences of giardial infections have been reported in many clinical series of immunodeficiency syndromes [86][87][88][89][90][91][92][93][94][95]. Whether reduced intestinal levels of immunoglobulins predispose to giardiasis in individuals without frank immunodeficiency syndromes is a matter of controversy.…”