There are two types of this disease based on morphology and immunophenotype, with both involving the small intestine: type I, which is often related to celiac disease (classic EATL), comprises 80 to 90% of all EATL cases, histologically presents with pleomorphic large or medium-sized cells with prominent inflammatory niches, and is most often CD4- and CD8-negative, and type 2, which is a monomorphic epitheliotropic variant, known as MEITL due to a revised 2016 classification, and most often CD 8-positive [6,7,9]. Here, CD4 is linked to celiac disease.