A Case of Diffuse Nodular Lymphoid Hyperplasia (NLH) of Duodenum in a Patient with Selective IgA Deficiency and Helicobacter Pylori Infection

Abstract

Introduction: Nodular lymphoid hyperplasia (NLH) is characterized by the presence of multiple small nodules, between 2-10 mm in diameter, distributed more commonly in the small intestine, sometimes in the stomach and large intestine. It affects individuals with or without immunodeficiency and is associated with diseases such as common variable immunodeficiency, selective IgA deficiency (sIgAD), giardia, celiac disease and helicobacter pylori (H pylori) infections. We present a case of diffuse duodenal NLH in a patient with sIgAD and H Pylori infection. Case: A 59 year old female with history of sIgAD presents with generalized weakness for 2 months. Hemoglobin at presentation was 9.1 g/dL (12-16 g/dL), IgA levels were 6.1 mg/dL (68-423 mg/dL). She denied melena, hematemesis or GI bleed. She underwent colonoscopy and endoscopy for normochromic normocytic anemia. Colonoscopy was unremarkable. Upper endoscopy showed nodular mucosa in the entire duodenum (figure 1), erythema in gastric antrum, and a single 7 mm sessile polyp in gastric body (figure 2). Pathology was consistent with NLH in duodenum (figure 3), gastric adenomatous polyp with low grade dysplasia and positive H pylori. She was started on treatment with clarithromycin, amoxicillin and pantoprazole. She underwent upper endoscopy with endoscopic ultrasound for resection of gastric adenoma which again showed nodular mucosa in duodenum consistent with NLH.Figure 1Figure 2Figure 3Discussion: The incidence of NLH is unknown but rare condition in adults. Histologically it is defined as hyperplastic, mitotically active germinal centers with well-defined lymphocyte mantles in lamina propria. In immunodeficiency states NLH may result from an accumulation of plasma-cell precursors due to a maturational defect in the development of B-lymphocytes. In H pylori infection, pathogenesis involves immune stimulation by H pylori. It is usually asymptomatic but can present with abdominal pain, chronic diarrhea, bleeding, intussusception or intestinal obstruction. The diagnosis is established by endoscopy or contrast barium studies and confirmed histologically. Treatment involves treating the associated conditions. NLH is a risk factor for both intestinal and extra-intestinal lymphomas. Some authors recommend surveillance, however, the duration and intervals are undefined. Conclusion: The case illustrates the rare impressive endoscopic findings of NLH in a patient with sIgAD and H. pylori infection.