A 45-year-old woman with celiac disease undergoes upper GI endoscopy. Duodenal biopsy shows Marsh Grade 3c changes (complete villous atrophy). She has been on a strict gluten-free diet for 12 months but remains symptomatic with diarrhea and weight loss. Which complication should be MOST suspected?
- A Microscopic colitis
- B Refractory celiac disease type 2 with risk of enteropathy-associated T-cell lymphoma (EATL) ✓
- C Collagenous sprue with secondary lactose intolerance
- D Small bowel bacterial overgrowth (SIBO)
Explanation
Refractory celiac disease (RCD) is defined as persistent villous atrophy and malabsorption despite strict gluten-free diet for > 12 months. RCD Type 2 has clonal expansion of aberrant intraepithelial lymphocytes lacking surface CD3/CD8 but expressing intracytoplasmic CD3—this carries a 50–80% risk of transformation to enteropathy-associated T-cell lymphoma (EATL), a rare but highly aggressive lymphoma. SIBO is a common cause of symptom persistence but would not explain continued Marsh 3c on biopsy. Microscopic colitis presents with watery diarrhea but normal small bowel mucosa. RCD Type 2 requires immunosuppression (budesonide, cladribine) and surveillance.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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