<unk>  <unk> , also<unk> -type T-cell lymphoma (ETTL), is a type of T-cell non-hodgkin lymphoma that affects the small intestine, it is composed of large lymphoid<unk> s.<unk>  has two subtypes, type I<unk>  which has a strong association with celiac disease and it is more common in western countries and type II<unk>  which is mostly found among the Asian population. Genes involved in the pathogenesis of this disease include 8q24, T-cell receptor (TCR) beta and gamma, and 16q genes. On gross pathology, multiple intestinal ulcers are characteristic findings of<unk> . On microscopic histopathological analysis, monotonous<unk> s, round or angulated vesicular nuclei, and prominent nucleoli are characteristic findings of<unk> . There are no established causes for<unk> .<unk>  must be differentiated from other diseases such as peptic ulcer, poorly-differentiated adenocarcinoma, MALT lymphoma, diffuse large B<unk>  lymphoma, and mantle<unk>  lymphoma. The incidence of<unk>  is very low worldwide. There are no established risk factors for<unk> .  Common complications of<unk>  include ulcer, obstruction, and perforation of small intestine. Prognosis is generally poor. According to the Lugano classification, there are four stages of<unk>  based on the number of nodes and extranodal involvement. The most common symptoms of<unk>  include fever, weight loss, skin rash, night sweats, chest pain, abdominal pain, bone pain, and painless swelling in the neck, axilla, groin, thorax, and abdomen. Common physical examination findings of<unk>   include fever, rash, ulcer, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy. Lymph node or endoscopic tissue biopsy is diagnostic of<unk> . CT scan may be helpful in the diagnosis of<unk> . Findings on CT scan suggestive of<unk>  include bowel wall thickening, mesenteric fat infiltration, mesentric lymph node cavitation, intussusception, and small-sized spleen. Other diagnostic studies for<unk>  include bone marrow aspiration and bone marrow biopsy. There is no treatment, the mainstay of therapy is supportive care. The optimal therapy depends on the extent and the location of the lymphoma in the small intestine.  Surgery is not the first ­line treatment option for patients with<unk> . Local debulking is usually reserved for patients with tumor masses with a high risk of obstruction, hemorrhage, and perforation
Enteropathy-associated T-cell lymphoma
MALT lymphoma
Gastric lymphoma
AIDS-related lymphoma
Celiac disease
Splenic marginal zone lymphoma
Hepatosplenic T cell lymphoma
Faisalabad histiocytosis