ENTEROPATHY-TYPE INTESTINAL T-CELL LYMPHOMA (EITCL) / ENTEROPATHY-ASSOCIATED T-CELL LYMPHOMA (EATCL / EATL) / MALIGNANT HISTIOCYTOSIS OF THE INTESTINE is the most common primary gastrointestinal T-cell lymphoma (< 1% of all NHLs). This is a clinically aggressive tumor that arises from the intestinal intraepithelial CTL population

Epidemiology : most patients are middle-aged to elderly males with a median age of 50 yearsref
Aetiology : can complicate an established history of gluten-sensitive enteropathy (celiac disease) but most often occurs following a short history of celiac disease and/or dermatitis herpetiformis (Jaffe ES, Railfkiaer E. Mature T-cell and NK neoplasms: introduction. In: Jaffe ES, Harris NL, Stein H, Vardiman JW, eds World Health Organization Classification of Tumours: Pathology and Genetics of Tumours of Hematopoetic and lymphoid Tissues. IARC Press: Lyon; 2001:191194). Even in the absence of a known history of celiac disease, there is usually histologic evidence of villous atrophy and crypt hyperplasia in the adjacent bowel when the tumor is excised. In the past several years the understanding of celiac-associated lymphoma has improved. Isaacson and Wrightref have shown that celiac-associated lymphomas were of single histogenetic type and probably derived from histiocytes. Subsequent immunohistochemical and molecular studies indicated that these tumors are of T-cell originref1, ref2. Wright et alref later described a patient with CD, jejunal ulcerations, and a clonal rearrangement of the TCRg and TCRb chain gene together with karytotype abnormalities of jejunal T cells. From this case report, they hypothesized that adult-onset CD could be a low-grade lymphoma of intraepithelial T lymphocytesref. However, other studies performed on isolated gd T-cell clonesref or fresh biopsy materialref from duodenojejunal mucosa of CD patients have found no evidence of the expansion of one or more T-cell clones in CD. It may be suggested that monoclonal T cells arose from a polyclonal infiltrate after long years of untreated, asymptomatic CDref.23 Studies performed in human normal colon and jejunum have shown oligoclonality of IEL bearing  phenotyperef1, ref2, ref324-26 and  TCR-bearing cellsref1, ref2.27,28 It was thus not unexpected that some patients with uncomplicated CD and controls had an oligoclonal pattern. However, in patients with complicated CD, a dominant clone was evidenced, indicating a preferential expansion of a T-cell clone among a highly cellular inflammatory mucosa
Symptoms & signs :

Laboratory examinations : Therapy : partial small bowel resection. Nutritional support is critical in the management of these patients. Treatment is often complicated by poor nutrition and significant risk of bowel perforation. Prognosis : most patients die within 10 months after diagnosis; median survival of 7.5 months and < 20% failure-free survival at 1 yearref1, ref2

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