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. 2011 Feb 3;117(5):1447-52.
doi: 10.1182/blood-2010-08-302737. Epub 2010 Oct 21.

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

Adnan Mansoor  1 , Stefania Pittaluga, Paul L Beck, Wyndham H Wilson, Judith A Ferry, Elaine S Jaffe
Affiliations

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

Adnan Mansoor et al. Blood. .

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.

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Figures

Figure 1
Figure 1
Endoscopic images of NK-cell enteropathy. (A-B) Colonic lesions. The colonic lesions generally appeared as ulcers with surrounding erythema and edema (A), but some lesions had a more nodular or polypoid appearance (B). (C-D) Gastric and duodenal lesions. The main lesions noted were raised ulcer-like lesions surrounded by erythema and edema (duodenal lesion; C); a few small punctuate deep ulcers in the stomach (D) were also noted and had similar pathologic appearance as the other NK-cell lesions throughout the gastrointestinal tract. Images were processed with Adobe Photoshop.
Figure 2
Figure 2
Mucosal biopsy findings. (A) Biopsy of the gastric antrum shows diffuse infiltration of the lamina propria by the atypical cells. The muscularis mucosa is intact, without infiltration of the submucosa. (B) A CD56 stain of the same biopsy highlights the atypical infiltrate. Photomicrographic images were acquired with a Nikon Eclipse 50i microscope equipped with an Olympus DP71 camera and software. Final image preparation was performed with Adobe Photoshop CS3 extended Version 10.0.1. Magnification: 2×/0.1 NA Plan Apo lens.
Figure 3
Figure 3
Histologic and immunohistochemical features of NK-cell enteropathy. Representative cases are shown involving stomach (A-D), duodenum (E), and colon (F). (A) The atypical cells infiltrate the mucosa, separating and displacing the glands. (B) Epitheliotropism is largely absent, but the cells show cytologic atypia, with irregular nuclei and eosinophilic, granular cytoplasm. (C) NK cells are strongly positive for CD56 and diffusely infiltrate the mucosa, with rare cells infiltrating the glandular epithelium. The infiltrate does not extend to the submucosa. (D) The cells express cytoplasmic CD3 (D), and immunohistochemical stains highlight the cytologic atypia. (E) CD56 identifies atypical NK cells diffusely infiltrating and expanding duodenal villi. (F) Atypical cells surround colonic glands with focal areas of superficial hemorrhage. (C-E) Immunohistochemistry with avidin-biotin-peroxidase complex immunoperoxidase technique and hematoxylin counterstain. Photomicrographic images were acquired with a Nikon Eclipse 50i microscope equipped with an Olympus DP71 camera and software. Final image preparation was performed with Adobe Photoshop CS3 extended Version 10.0.1. Original magnifications: panel A, 4×/0.2 NA; panel B, 40×/0.95 NA; panel C, 10×/0.45 NA; panel D, 100×/1.40 NA; panel E, 10×/0.45 NA; panel F, 20×/0.75 NA.
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Comment in

  • A sheep in wolf's clothing.
    Porcu P, Caligiuri M. Porcu P, et al. Blood. 2011 Feb 3;117(5):1438-9. doi: 10.1182/blood-2010-11-319913. Blood. 2011. PMID: 21292781

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