Diffuse large B-cell lymphoma of the small intestine in a patient with refractory coeliac disease
- Author:
Khairunisa Ahmad Affandi
1
Author Information
1. Department of Pathology, Universiti Kebangsaan Malaysia Medical Centre, Kuala Lumpur, Malaysia
- Collective Name:Khairunisa Ahmad Affandi; Nordashima Abd Shukor; Isa Mohamed Rose; Raja Affendi Raja Ali; Noraidah Masir
- Publication Type:Case Reports
- Keywords:
Diffuse large B-cell lymphoma;
refractory coeliac disease;
small intestine;
perforation
- MeSH:
refractory coeliac disease
- From:The Malaysian Journal of Pathology
2019;41(1):65-69
- CountryMalaysia
- Language:English
-
Abstract:
Introduction: Coeliac disease enteropathy is associated with an increased risk of lymphomas. Enteropathy-associated T-cell lymphoma is the principal malignancy related to coeliac disease. However, studies have shown that other types of lymphoma such as diffuse large B-cell lymphoma may also be associated with coeliac disease. Case Report: We report a 54-year-old Caucasian man who presented with chronic diarrhoea and weight loss. He was diagnosed with coeliac disease based on positive serology results and duodenal, jejunal, and ileal biopsies that showed villous atrophy. Despite adherence to a gluten-free diet, there was no clinical remission and enteropathy-associated T cell lymphoma was suspected. Repeated endoscopic biopsy showed persistent mucosal disease but no evidence of lymphoma. Several weeks later he presented with a perforated jejunum. Histology of the resected jejunum showed diffuse infiltration of submucosa and muscularis propria by malignant lymphoid cells sparing the mucosa. The cells expressed CD20, CD79α, CD10 and BCL6 and ki67 of 80%, consistent with diffuse large B-cell lymphoma. Discussion: It is suspected that the undetected lymphoma may have contributed to the persistent malabsorption syndrome rendering the patient unresponsive to treatment. Despite thorough clinical and endoscopic evaluation and multiple biopsies, histologic diagnosis of DLBCL was only confirmed following resection of the perforated jejunum.
- Full text:5.2019my0995.pdf
