[DISCUSSION]
Enteropathy-associated T-cell lymphoma (EATL) is a rare form of intraepithelial T-cell intestinal lymphoma (1). The annual incidence rate is 0.5–1 per million people in Western countries and this is a rare form of malignancy, covering approximately 35% of all small bowel lymphomas (1). The pathogenesis of EATL is multifactorial, involving chronic inflammation and immune dysregulation within the small intestine, particularly in individuals predisposed to celiac disease. Most commonly affected area is small intestine (jejunum> duodenum/ileum) (7). Even though this kind of lymphoma is uncommon, it is one of the leading causes of mortality for adults with celiac disease (CD) (8).
In 1937, Farley and Mackie initially described the association between intestinal lymphoma and malabsorption (9). O’Farrelly coined the term ‘Enteropathy associated T cell lymphoma’ in 1986, establishing the close relationship between this lymphoma with villous atrophy of the jejunal mucosa adjacent to EATL (10).
World health organization(WHO) divided enteropathy associated t cell lymphoma(EATL) into two subtypes in 2008, into EATL type I and EATL Type II (11). EATL type I is typically associated with refractory celiac disease, which accounts for 80-90% of cases, and often presents with large-cell or pleomorphic cytology, with infrequent expression of CD8 and CD56 (12). While EATL type II, constituting 10-20% of cases, is sporadic, less commonly associated with celiac disease, and characterized by monomorphic cytology with frequent expression of CD8 and CD56 (13). In 2016 WHO redefined two diseases as, EATL Type I as EATL, and EATL type II as Monomorphic epithelia-tropic intestinal t cell lymphoma(MEITL) (14). EATL is 5-10 times more common than MEITL (15).
The common age group for EATL is 60’s and 70’s with similar prevalence in both genders (16). EATL most commonly develops in the jejunum but it can also develop in other parts of small intestine and colon (17). It may also spread to spleen, stomach, lymph node, liver and gall bladder (18). Despite its primary localization in the small intestine, EATL manifesting in the stomach, poses diagnostic challenges as it may mimic other gastric lesions such as peptic ulcers, leading to diagnostic confusion. Accurate diagnosis is crucial as EATL requires distinct management strategies compared to other gastric malignancies. Affected patient can present with similar symptoms of celiac disease like abdominal pain/distention, malabsorption, weight loss, night sweats, chronic recurrent diarrhea. Sometimes it can suddenly develop with serious symptoms of bowel perforation and/or bowel obstruction (16,19).
The atypical presentation of EATL as acute abdominal pain without classical symptoms underscores the need for a comprehensive differential diagnosis in emergency settings. While gastrointestinal perforation is an uncommon manifestation of lymphomas (20), this case emphasizes the importance of a timely surgical approach for both diagnosis and therapeutic intervention. Despite advancements in diagnostic techniques and treatment modalities, EATL remains a challenging entity to manage due to its rarity and heterogeneous clinical presentation. Therefore, increased awareness among healthcare professionals regarding the association between celiac disease and EATL is paramount for timely diagnosis and appropriate management. The exact mechanisms underlying the development of EATL remain poorly understood, highlighting the need for further research in this.