Enteropathy-associated T-cell lymphoma without a prior diagnosis of coeliac disease: diagnostic dilemmas and management options

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Abstract
Enteropathy-associated T-cell lymphoma (EATL) ultimately develops in 7–10% of patients with long-standing coeliac disease. In patients without a prior diagnosis of coeliac disease this is a very rare disorder, and the diagnosis in such cases is often difficult and delayed due to the non-specific nature of the symptoms and a very low index of clinical suspicion. Standard anti-lymphoma therapies have minimal utility in patients with EATL, and their prognosis is poor. An added difficulty is the high risk of intestinal perforation especially with the commencement of treatment due to the multifocal nature of bowel disease and poor underlying nutrition and tissue integrity. To illustrate these problems and provide an example of how these issues may be addressed, we report the case of a patient with EATL who was completely asymptomatic from unsuspected underlying coeliac disease and presented initially with back pain followed by bowel obstruction. He was treated with gut rest with total parenteral nutrition before commencing an intensive chemotherapy regimen [hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, and dexamethasone)] and is currently well in ongoing complete remission 34 months later.