Gastric form of alpha chain disease.

Abstract

A case of alpha chain disease, involving the stomach only, is reported in an Algerian man suffering from epigastric pains. Upper digestive tract fibreoptic endoscopy showed two antral ulcers and an ulcerative gastritis pattern, which promptly disappeared with cimetidine treatment. Antral biopsies at a distance from the ulcers, but not of the ulcer crater itself, disclosed a dense infiltration of antral lamina propria by mature or sometimes atypical plasma cells. On transmural surgical antral biopsy, the infiltrate spread to the superficial part of the submucosa. No other localisation of the disease was found in spite of multiple biopsies obtained by endoscopy, with a peroral capsule and during staging laparotomy. The alpha chain disease protein was absent from serum and urine, but found in the gastric juice and in the cytoplasma of the cellular infiltrate (al subclass). A complete clinical, endoscopic, histological and immunological remission was observed after a six months' course of oral tetracycline. Nearly all reported cases of alpha chain disease were mainly localised to the small intestine and usually mesenteric lymph nodes. 1-3 Very few cases apparently sparing the small bowel have been described,-9 but in none of them the definite evidence of small intestinal integrity was obtained. We report here a well documented case of exclusive gastric involvement in alpha chain disease. The presence of the abnormal IgAl in the gastric juice, but not in the serum, and the complete remission obtained with antibiotic treatment alone are other points of interest.

Immunoproliferative small intestinal disease (IPSID): a model for mature B-cell neoplasms

“…This is not withstanding the fact that there are rare patients with ␥ rather than ␣ heavy chain IPSID 9 and the rare colonic, gastric, or pulmonary ␣HCD. [10][11][12] IPSID is a unique mature B-cell neoplasm regarding its epidemiology, clinical features, morphology, and molecular pathogenesis. 13 It shares certain features with gastrointestinal MALT lymphomas, lymphoplasmacytic lymphoma, as well as plasma cell neoplasms.…”

Gastrointestinal lymphoma

“…Most cases of IPSID with AHC involvement occur in the small intestine although cases of gastric and colonic involvement have been reported [95,96]. The entire small intestine is usually involved with the areas of highest concentration being the jejunum and duodenum [97,98].…”

Gastric Alpha Heavy Chain Disease

“…When this manuscript was ready for submission, the authors' attention was drawn to another case report of gastric form of AHCD [31]. The pathological and immunohistological studies in this case were made on endoscopic and limited surgical biopsy specimens.…”