Extranodal NK/T-cell lymphoma, nasal type accompanied by PR3-ANCA-associated glomerulonephritis

Yuta Tamoto, Ryo Ishida, Kazuya Shiogama, Hiroshi Kado, Michitsugu Kamezaki, Yoshiaki Chinen, Yosuke Matsumoto, Tetsuro Kusaba, Eiichi Konishi, Yutaka Tsutsumi, Keiichi Tamagaki

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Abstract

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.

Original languageEnglish
Pages (from-to)2007-2012
Number of pages6
JournalInternal Medicine
Volume56
Issue number15
DOIs
Publication statusPublished - 01-01-2017

All Science Journal Classification (ASJC) codes

  • Internal Medicine

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    Tamoto, Y., Ishida, R., Shiogama, K., Kado, H., Kamezaki, M., Chinen, Y., Matsumoto, Y., Kusaba, T., Konishi, E., Tsutsumi, Y., & Tamagaki, K. (2017). Extranodal NK/T-cell lymphoma, nasal type accompanied by PR3-ANCA-associated glomerulonephritis. Internal Medicine, 56(15), 2007-2012. https://doi.org/10.2169/internalmedicine.56.8365