A 40-year-old woman with long-standing coeliac disease maintained on a strict gluten-free diet presents with persistent diarrhoea, weight loss, and CT showing thickening of multiple jejunal loops with mucosal ulceration. Small bowel biopsy shows persistent villous atrophy and intraepithelial T-lymphocytes. Which complication is most likely?
- A Refractory coeliac disease type II progressing to enteropathy-associated T-cell lymphoma (EATL) ✓
- B Gluten re-exposure causing classical relapse
- C Collagenous sprue due to medication
- D Tropical sprue with concurrent infection
Correct answer: A. Refractory coeliac disease type II progressing to enteropathy-associated T-cell lymphoma (EATL)
Explanation
Refractory coeliac disease type II is characterised by persistent villous atrophy despite strict gluten-free diet with a clonal population of aberrant intraepithelial lymphocytes (lacking surface CD3/CD8). It carries a high risk of transformation to enteropathy-associated T-cell lymphoma (EATL type I), a highly aggressive peripheral T-cell lymphoma with poor prognosis. CT showing ulcerative jejunitis is a key radiological finding. Classic relapse improves with dietary restriction, unlike RCD-II.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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