NK-cell enteropathy: a benign NK-cell lymphoproliferative disease mimicking intestinal lymphoma: clinicopathologic features and follow-up in a unique case series
NK-cell enteropathy: a benign NK-cell lymphoproliferative disease mimicking intestinal lymphoma: clinicopathologic features and follow-up in a unique case series
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DOI：
10.1182/blood-2010-08-302737
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发表时间：
2011-02-03
期刊：
影响因子：
20.3
通讯作者：
Jaffe, Elaine S.
中科院分区：
文献类型：
--
作者：
Mansoor, Adnan;Pittaluga, Stefania;Jaffe, Elaine S.
肠道T细胞和自然杀伤（NK）细胞淋巴瘤在临床上具有侵袭性，在小型内镜活检中诊断具有挑战性。我们描述了8例非典型NK细胞淋巴增生性病变模仿NK细胞或T细胞淋巴瘤。患者（2名男性; 6名妇女;年龄27-68岁）表现出模糊的胃肠道症状，病变累及胃、十二指肠、小肠和结肠。内窥镜检查发现病变表现为浅表溃疡、水肿和出血。活检显示具有NK细胞表型（CD 56（+）/TIA-1（+）/颗粒酶B+/cCD 3（+））的非典型细胞的粘膜浸润，其移位但未侵入腺上皮。EB病毒编码的RNA原位杂交呈阴性，T细胞受体-γ基因重排显示没有克隆过程的证据。3例患者最初诊断为淋巴瘤，接受了积极的化疗，2例接受了自体骨髓移植。5例患者未接受治疗。然而，没有患者发生疾病进展或死于淋巴瘤（中位随访时间为30个月）。8例患者中有6例重复内镜检查显示浅表胃肠道病变持续存在或复发。这种独特的实体在内窥镜活检中模仿肠道和NK/T细胞淋巴瘤，可能导致误诊，导致积极的化疗。我们提出术语“NK细胞肠病”，这种综合征的病因尚未明确。（血。2011; 117（5）：1447-1452）
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-gamma 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. (Blood. 2011; 117(5): 1447-1452)