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Type II refractory sprue, collagenous sprue
van Gieson
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.
Blunting of villi. Dense interstitial lymphocytic infiltration. Sub-epithelial collagenous band with loss of epithelial layer (thickness > 30 um) with entrapment of cells. No sign of crypt hyperplasia but focal crypt atrophy. Sparse mitotic activity for celiac disease.
For HE stain see slide ID: 1541 For CD3 IHC see slide ID: 1543 For CD8 IHC see slide ID: 1544 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 in this case 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). Collagenous sprue is a rare form of small bowel enteropathy characterized by chronic diarrhea and progressive malabsorption with little data available on its natural history. The occurrence of collagenous sprue has been reported in individuals with celiac disease, tropical sprue, milk intolerance, myotonic dystrophy, malignancy-related paraneoplastic syndrome, and common variable immunodeficiency. The pathologic features consist of sub-epithelial collagen deposition with entrapment of cells and variable alterations in villous architecture.
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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