A 30-year-old woman with refractory celiac disease (persistent villous atrophy despite 12 months of strict gluten-free diet) is evaluated for enteropathy-associated T-cell lymphoma. Which immunophenotype is characteristic of the lymphoma cells in type II refractory celiac disease?

• A CD3+, CD4+, CD8+, CD30+
• B CD3+, CD4-, CD8-, CD30- ✓
• C CD3+, CD4-, CD8+, CD30+
• D CD3-, CD4-, CD8-, CD30+

Explanation

Type II refractory celiac disease is characterized by an aberrant intraepithelial lymphocyte population that is CD3 positive but lacks CD4 and CD8 (double negative) and is CD30 negative. This clonal population is a low-grade lymphoma that can transform into enteropathy-associated T-cell lymphoma (EATL). Type I RCD has normal lymphocyte immunophenotype. CD30 positivity is seen in EATL but not in the precursor aberrant cells of type II RCD.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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