Refractory Celiac Disesae (RCD) can be divided into 2 categories based on the cellular immune response.
Criteria for refractory celiac disease:
(1) persistent villous atrophy with crypt hyperplasia
(2) increased intra-epithelial T cell lymphocytes (IEL)
(3) (despite strict gluten-free diet (GFD) for > 12 months) OR (intervention required for severe symptoms)
Type
Features of IELs
Prognosis
RCD I
expression of T-cell surface markers not aberrant
fair with 5-year survival of 93%
RCD II
aberrant expression of T-cell surface markers
poor with increased risk of malignancy, poor response to therapy and 5-year survival of 44%
Aberrant T-cells in RCD-II may show:
(1) loss of normal surface markers CD3, CD4 and CD8 with preserved intracytoplasmic CD3 (CD3e) in > 50% of IEL by immunohistochemistry or > 20% by flow cytometry
(2) clonal rearrangement of T-cell receptor chains (delta or gamma) by PCR.
Malignancies associated with RCD II:
(1) enteropathy-associated T-cell lymphoma (EATL)
(2) other lymphoma
(3) gastrointestinal adenocarcinoma
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