Enteropathy-associated T-cell lymphoma (EATCL)

ENTEROPATHY-TYPE T-CELL LYMPHOMA / 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 years^ref
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; 2001:191–194). 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.
Symptoms & signs :

diet-refractory coeliac disease
ulcerating jejunitis
rarely anasarca
small bowel perforation or obstruction is common and the diagnosis of enteropathy-type intestinal T cell lymphoma is usually made at laparotomy
blood and tissue hypereosinophilia due to IL-5 production by tumor cells
abdominal pain and weight loss occur in > 80% of patients at presentation
diarrhea or vomiting in approximately one-third of patients^ref
although most patients have clinical stage I or IIE disease, dissemination to the liver, spleen, lung, skin, and bone marrow can occur

Laboratory examinations : immunophenotype CD3⁺4^–7⁺8^-103⁺, TcR^-
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.

CHOEP is not effective^ref

Prognosis : most patients die within 10 months after diagnosis; median survival of 7.5 months and < 20% failure-free survival at 1 year^ref1,
ref2

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