The patient is a 69-year-old man who has clinical history of celiac disease and elevated tissue transglutaminase IgA of >123 units (normal <20 units). Reportedly the patient was not following gluten-free diet at the time of multiple upper GI endoscopic biopsies performed confirming the diagnosis of Coeliac disease. Subsequent resection of a segment of a jejunal mass was performed whic showed Enteropathy associated T-cell lymphoma.

EATL is divided into two subtypes: EATL Type I occurs typically in people of European ancestry with a strong Coeliac disease association. The tumor cells are pleomophic with variable expression of surface T-cell receptors and cytotoxic markers. The case depicted below was however negative for both TCRs (silent/null phenotype) which can occur in a small proportion of cases.

EATL, Type II on the other hand is typically positive for CD8 and CD56 and has been observed more frequently in Asian and Hispanic populations with increasingly frequent gamma-delta TCR expression as opposed to most EATL, Type I. Moreover, recent identication of underlying STAT5B mutations in nearly 36% of cases designated EATL Type II (all expressing TCR gamma/delta) has led to the new designation of EATL Type II as 'monomorphic epithliotropic Intestinal T-cell lymphoma' (MEITL) in the 2016 WHO classification.