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Type II refractory sprue, collagenous sprue
70 years
First diagnosis of celiac disease 4 years prior to current biopsy. Negativity for anti-transglutaminase antibodies under strict gluten free diet, but persistent weight loss.oss.
The majority of the intraepithelial lymphocytes are negative for CD8. Blunting of villi. Dense interstitial lymphocytic infiltration. Sub-epithelial collagenous band with loss of epithelial layer (thickness > 30 um) and entrapment of cells. No sign of crypt hyperplasia but focal crypt atrophy.
For HE stain see slide ID: 1541 For EvG stain see slide ID: 1542 For CD3 IHC see slide ID: 1543 No proof of monoclonal T-cell receptor rearrangement in the performed PCR.
The morphological aspect corresponds to a collagenous sprue. The majority of the intraepithelial lymphocytes are positive for CD3 and negative for CD8. This phenotype is compatible with a refractory sprue type II. These lesions are considered by a majority of the authors as a 'lymphoma in situ'. The prognosis is therefore often bad (see also the indicated reference).
De Mascarel A, Belleannée G, Stanislas S, Merlio C, Parrens M, Laharie D, Dubus P, Merlio JP.
Mucosal intraepithelial T-lymphocytes in refractory celiac disease: a neoplastic population with a variable CD8 phenotype.
Am J Surg Pathol. 2008 May;32(5):744-51. doi: 10.1097/PAS.0b013e318159b478. PubMed PMID: 18360280.
Link: http://www.ncbi.nlm.nih.gov/pubmed/1836028060280
Joël Gsponer
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