Therefore, in a patient with chronic, intractable GI tract symptoms

but with no evidence of gastric or colonic disease on upper and lower endoscopy, diseases of the small intestine (e.g., malignancy, #TG101348 manufacturer randurls[1|1|,|CHEM1|]# infection, autoimmune disease) should be considered and evaluated. Technics such as video capsule endoscopy, double-balloon enteroscopy, computed tomography (CT) scan combined Inhibitors,research,lifescience,medical with 18F fluorodeoxyglucose positron emission tomography (18F-FDG-PET) scan and magnetic resonance enteroclysis are possible modalities to investigate small intestinal malignancy (9). Prevention of EATL may be feasible in some cases with early diagnosis of CD and adherence to a strict gluten-free diet, which can lead to a four-fold reduction in the risk of EATL in CD, compared to patients Inhibitors,research,lifescience,medical who are non-compliant (7). Even compliant CD patients need monitoring for progression to refractory CD (RCD). RCD is divided into two types- type I is characterized by persistent or recurrent symptoms, positive CD-specific serology, and/or villous atrophy after 6-12 months on a gluten free diet and exclusion of other etiologies (10). Inhibitors,research,lifescience,medical RCD type II is diagnosed when an abnormal (clonal) population of intraepithelial T-cells is also present. These clonal T-cells show loss of normal surface markers CD3, CD4 and CD8

with preserved expression of intracytoplasmic CD3 in >50% by immunohistochemistry (or >20-25% by flow cytometry). These abnormal T-cells may also be present Inhibitors,research,lifescience,medical in lamina propria (11). 60-80% of patients with RCD type II will progress to EATL (12). Endoscopically, RCD type II shows either multiple ulcers (“ulcerative jejunitis”) or large ulcers (>1 cm). The presence of nodules, masses and strictures, as well as cytologic atypia, suggest progression to EATL (10). RCD type II is associated with a 5-year survival rate of only 40-58% (10), but survival may be improved with high-dose

chemotherapy and autologous stem-cell transplantation before development of EATL (12). The pathological diagnosis of EATL has potential pitfalls as well. In Inhibitors,research,lifescience,medical EATL type I, the lymphocytes are medium-sized to large cells with round or angulated vesicular nuclei, prominent nucleoli and moderate to abundant, pale-staining cytoplasm (5). Less often, Quizartinib the tumor cells are more pleomorphic and sometimes multi-nucleated, resembling anaplastic large cell lymphoma. EATL type I tends to be infiltrated with abundant eosinophils and histiocytes. Coagulative necrosis is common. The intestinal mucosa adjacent to the primary tumor frequently shows enteropathy with villous atrophy, crypt hyperplasia, increased inflammatory cells in the lamina propria, and intraepithelial lymphocytosis (2). In contrast, EATL type II is characterized by multiple foci of small round uniform cells, with dark nuclei and a rim of pale cytoplasm.