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1 Distinctive morphologic features of extranodal NK-/T-cell lymphoma are an angiocentric and angiodestructive growth pattern with frequent necrosis and apoptosis. We propose the term “NK-cell enteropathy” for this syndrome of as yet unknown etiology.Įxtranodal natural killer (NK)-/T-cell lymphomas are postulated to originate from activated NK cells, although some cases have been shown to have a true T-cell derivation. This unique entity mimics intestinal and NK-/T-cell lymphomas on endoscopic biopsies and can result in erroneous diagnosis, leading to aggressive chemotherapy.
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Repeat endoscopies in 6 of 8 patients showed persistence or recurrence of superficial gastrointestinal lesions. However, no patient developed progressive disease or died of lymphoma (median follow-up, 30 months). Five patients were followed without treatment. Based on an original diagnosis of lymphoma, 3 patients received aggressive chemotherapy followed by autologous bone marrow transplantation in 2. Epstein-Barr virus–encoded RNA in situ hybridization was negative, and T-cell receptor-γ gene rearrangement showed no evidence of a clonal process. Biopsies revealed a mucosal infiltrate of atypical cells with an NK-cell phenotype (CD56 +/TIA-1 +/Granzyme B +/cCD3 +), which displaced but did not invade the glandular epithelium. At endoscopy, the lesions exhibited superficial ulceration, edema, and hemorrhage. The patients (2 men 6 women ages 27-68 years) presented with vague gastrointestinal symptoms with lesions involving stomach, duodenum, small intestine, and colon. We describe 8 patients in whom atypical NK-cell lymphoproliferative lesions mimicked NK- or T-cell lymphoma. Intestinal T-cell and natural killer (NK)–cell lymphomas are clinically aggressive and can be challenging to diagnose in small endoscopic biopsies.