A 45-year-old man with celiac disease (confirmed on duodenal biopsy — villous atrophy, crypt hyperplasia, intraepithelial lymphocytosis, Marsh III) develops worsening diarrhea and weight loss despite strict gluten-free diet for 2 years. Repeat biopsy shows persistent villous atrophy and intraepithelial lymphocytes. The most important condition to exclude is:
- A Inadvertent dietary gluten contamination
- B Collagenous sprue (collagen band >10 µm under epithelium)
- C Small intestinal bacterial overgrowth (SIBO) as a complication
- D Refractory celiac disease type II (RCD-II) progressing to enteropathy-associated T-cell lymphoma (EATL) ✓
Explanation
Refractory celiac disease (RCD) is defined as persistent malabsorption with villous atrophy despite strict gluten-free diet for >12 months, after excluding dietary gluten contamination and other causes. RCD type II has aberrant intraepithelial T-lymphocytes (sIgE−, intracellular CD3+, CD8−) representing a clonal T-cell population — this carries a high risk (50–80%) of progression to enteropathy-associated T-cell lymphoma (EATL) within 5 years, which is an aggressive lymphoma with poor prognosis. Dietary contamination must be excluded first (most common cause), but RCD-II requires bone marrow/PET-CT evaluation and oncology referral. Flow cytometry of IEL population is diagnostic.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.