A 62-year-old man with coeliac disease on a strict gluten-free diet for 2 years has persistent diarrhoea and weight loss. Small bowel biopsy shows villous atrophy and increased intraepithelial lymphocytes (IEL > 40/100 enterocytes). Flow cytometry of IELs shows aberrant CD3+/CD8− (surface CD3 negative) phenotype. This finding suggests:
- A Dietary non-compliance; repeat dietary counselling
- B Type I refractory coeliac disease responding to steroids
- C Type II refractory coeliac disease with risk of enteropathy-associated T-cell lymphoma (EATL) ✓
- D Tropical sprue
Explanation
Refractory coeliac disease (RCD) type II is defined by aberrant clonal IELs with surface CD3-negative/intracytoplasmic CD3+/CD8-negative phenotype, representing a clonal T-cell expansion that is precancerous; it carries a 5-year risk of EATL (formerly Type I EATL) of approximately 50%. RCD Type I has normal IEL phenotype and responds to immunosuppressive therapy (corticosteroids, azathioprine). Type II RCD requires more aggressive treatment (cladribine, autologous SCT) and close surveillance. Dietary non-compliance would not explain aberrant IEL phenotype. Tropical sprue is a different entity without aberrant IELs.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.