NDLI: NK-cell enteropathy: a benign NK-cell lymphoproliferative disease mimicking intestinal lymphoma: clinicopathologic features and follow-up in a unique case series

Content Provider	PubMed Central
Author	Mansoor, Adnan Pittaluga, Stefania Beck, Paul L. Wilson, Wyndham H. Ferry, Judith A. Jaffe, Elaine S.
Abstract	Intestinal T-cell and natural killer (NK)–cell lymphomas are clinically aggressive and can be challenging to diagnose in small endoscopic biopsies. We describe 8 patients in whom atypical NK-cell lymphoproliferative lesions mimicked NK- or T-cell lymphoma. The patients (2 men; 6 women; ages 27-68 years) presented with vague gastrointestinal symptoms with lesions involving stomach, duodenum, small intestine, and colon. At endoscopy, the lesions exhibited superficial ulceration, edema, and hemorrhage. 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. Epstein-Barr virus–encoded RNA in situ hybridization was negative, and T-cell receptor-γ gene rearrangement showed no evidence of a clonal process. Based on an original diagnosis of lymphoma, 3 patients received aggressive chemotherapy followed by autologous bone marrow transplantation in 2. Five patients were followed without treatment. However, no patient developed progressive disease or died of lymphoma (median follow-up, 30 months). Repeat endoscopies in 6 of 8 patients showed persistence or recurrence of superficial gastrointestinal lesions. This unique entity mimics intestinal and NK-/T-cell lymphomas on endoscopic biopsies and can result in erroneous diagnosis, leading to aggressive chemotherapy. We propose the term “NK-cell enteropathy” for this syndrome of as yet unknown etiology.
Related Links	http://dx.doi.org/10.1182/blood-2010-08-302737
Ending Page	1452
Page Count	6
Starting Page	1447
File Format	PDF
ISSN	00064971
e-ISSN	15280020
Journal	Blood
Issue Number	5
Volume Number	117
Language	English
Publisher	American Society of Hematology
Publisher Date	2011-02-03
Access Restriction	Open
Rights Holder	American Society of Hematology
Subject Keyword	Immunology Cell Biology Biochemistry Hematology Research in Higher Education
Content Type	Text
Resource Type	Article
Subject	Cell Biology Hematology Biochemistry Immunology

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