Tamoto Yuta, Ishida Ryo, Shiogama Kazuya, Kado Hiroshi, Kamezaki Michitsugu, Chinen Yoshiaki, Matsumoto Yosuke, Kusaba Tetsuro, Konishi Eiichi, Tsutsumi Yutaka, Tamagaki Keiichi
Division of Nephrology, Department of Medicine, Kyoto Prefectural University of Medicine, Japan.
Department of Pathology, Fujita Health University School of Medicine, Japan.
Intern Med. 2017;56(15):2007-2012. doi: 10.2169/internalmedicine.56.8365. Epub 2017 Aug 1.
A 62-year-old man exhibiting nasal obstruction and glomerulonephritis with proteinase 3-antineutrophil cytoplasmic antibodies (PR3-ANCAs) was diagnosed with extranodal NK/T-cell lymphoma, nasal type (ENKL) with infiltration of neutrophils with apoptosis. Chemoradiotherapy reduced the tumor, improved the renal function, and decreased the PR3-ANCA levels. ANCA-positivity is observed in immunoinsufficient diseases, in which neutrophils lead to apoptosis and translocate intracellular granules, such as PR3, to the cell surface, triggering the production of ANCAs. In our case, the PR3-ANCA production was derived from the expression of PR3 on the cell surface of apoptotic neutrophils. This is the first report on ENKL describing the mechanism of ANCA development.
一名62岁男性，表现为鼻塞及伴有蛋白酶3-抗中性粒细胞胞浆抗体（PR3-ANCA）的肾小球肾炎，被诊断为鼻型结外NK/T细胞淋巴瘤（ENKL），伴有中性粒细胞浸润及凋亡。放化疗使肿瘤缩小，肾功能改善，PR3-ANCA水平下降。在免疫功能不全疾病中可观察到ANCA阳性，其中中性粒细胞发生凋亡并将细胞内颗粒（如PR3）转运至细胞表面，从而触发ANCA的产生。在我们的病例中，PR3-ANCA的产生源于凋亡中性粒细胞细胞表面PR3的表达。这是首篇描述ENKL中ANCA产生机制的报告。
