Enteropathy-associated T-cell lymphoma, presenting as diet-refractory coeliac disease

History and clinical findings: A 61-year-old woman in poor general health was admitted to hospital because of progressive diarrheo, flatulence, fatigue and weight loss. The patient had a history of coeliac disease with poor dietary compliance over many years. Hence, the present clinical deterioration was unresponsive to a gluten-free diet. Investigations: Laboratory results showed signs of inflammation and malabsorption. The endoscopy of the duodenum revealed a flattened mucosal architecture, as is typical for coeliac disease. Enteroscopy revealed many small jejunal lesions. Histological examination of mucosa showed typical signs of coeliac disease without definite signs of lymphoma. Immunhistochemical analysis showed atypical intraepithelial T-cells, while the molecular biological study revealed a monoclonal T-cell clone, both supporting the diagnosis of an enteropathy associated T-cell lymphoma (EATCL). Diagnosis and therapy: The patient was enrolled in a study on intestinal non-Hodgkin lymphomas and accordingly received 6 courses of CHOP-chemotherapy. Clinical and histological improvement has lasted for over a year. Conclusion: Patients with coeliac disease unresponsive to a gluten-free diet or with a deteriorating clinical condition may have an enteropathy-associated T-cell lymphoma. This can manifest itself in the form of an ulcerative jejunitis. In the early stages of disease, immunhistochemical and molecularbiological analyses may lead to the correct diagnosis.