[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.