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

Abstract

SUMMARY In vitro and in vivo lymphocyte function was studied in six patients with primary hypogammaglobulinaemia and nodular lymphoid hyperplasia (NLH) of the bowel. Lymphocyte transformation, numbers of circulating T Nodular lymphoid hyperplasia (NLH) of the bowel is a condition virtually confined to patients with primary immunodeficiency. It is extremely rare in children and is generally a complication affecting adults with primary common variable late onset hypogammaglobulinaemia. Six patients were found to have NLH in the course of screening for gastrointestinal complications and we have analysed in vitro and in vivo lymphocyte function in these patients to establish whether they have any characteristics in common. In addition, we have attempted to identify the cell types within the nodules. Methods SUBJECTSThirty-four patients with primary hypogammaglobulinaemia were screened for NLH. They were selected on the basis of their willingness to be inpatients and there was no tendency for patients with gastrointestinal complaints to be referred. Their ages at the time of investigation ranged from 7 to 63 years (mean 33 years). Seventeen patients were receiving regular weekly intramuscular gammaglobulin at a dose of 25-50 mg/kg body weight. Received for publication 2 March 1977Twenty-eight patients had grossly depressed levels of serum IgG-that is, <50 IU/ml-mean = 29 IU/mi) and only six patients had levels above 50 IU/ml. Serum IgA concentrations were unrecordable in 29 patients-that is, <3 5 IU/mland only one patient had a level within the normal limits. Twenty-eight patients had serum IgM concentrations below 47 IU/ml (mean = 12).

COEXISTENCE OF SYMPTOMATIC IRON-DEFICIENCY ANEMIA AND DUODENAL NODULAR LYMPHOID HYPERPLASIA DUE TO GIARDIASIS: Case Report

“…Common variable immunodeficiency (CVID) is observed most frequently in the group with immunodeficiency. Nearly 20% of the patients with CVID have NLH [3,4,11]. Similar findings have been reported in patients with isolated IgA deficiency and HIV [6,12].…”

“…Our patient was thought to have NLH without immunodeficiency because history of recurrent infections was absent and serum levels of immunoglobulins were normal. Gastrointestinal lesions in patients with CVID and NLH are mostly confined to the small intestine [6,11]. Distribution of the nodules in NLH patients without immunodeficiency tends to include the small intestine and colon [6,13].…”

Gastrointestinal Manifestations in Patients with Common Variable Immunodeficiency

“…It has been reported that 24.5% of CVID patients with chronic diarrhea have histological features of intestinal villous atrophy [11,14,16]. Nodular lymphoid hyperplasia of the small bowel is also frequently observed in patients with CVID [11][12][13][14][15][16][17][18]. These patients are also at increased risk for gastric adenocarcinoma and small-bowel lymphoma arising in the setting of nodular lymphoid hyperplasia [19].…”

Immunological Study in Primary Intestinal Lymphangiectasia

“…It is a frequent disorder [16] in young subjects, and often regresses spontaneously. Nonetheless, it cannot be ruled out that NLH is an indirect conse quence of the hypogammaglobulinemia [17,18]. This possible pathophysiological mechanism raises the ques tion of the development of NLH in PIL towards malig nant lymphomas, as it is known that lymphomas may complicate PIL.…”