Extranodal NK/T-cell Lymphoma, Nasal Type Accompanied by PR3-ANCA-associated Glomerulonephritis

Intern Med. 2017;56(15):2007-2012. doi: 10.2169/internalmedicine.56.8365. Epub 2017 Aug 1.

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.

Keywords: apoptosis; extranodal NK/T-cell lymphoma; nasal type; neutrophil; proteinase 3 antineutrophil cytoplasmic antibodies.

Publication types

  • Case Reports

MeSH terms

  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / etiology
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / immunology
  • Antibodies, Antineutrophil Cytoplasmic / blood*
  • Apoptosis / immunology
  • Diagnosis, Differential
  • Glomerulonephritis / etiology*
  • Glomerulonephritis / immunology
  • Granulomatosis with Polyangiitis / diagnosis
  • Humans
  • Lymphoma, Extranodal NK-T-Cell / complications*
  • Lymphoma, Extranodal NK-T-Cell / diagnosis
  • Lymphoma, Extranodal NK-T-Cell / immunology
  • Lymphoma, Extranodal NK-T-Cell / pathology
  • Male
  • Middle Aged
  • Myeloblastin / immunology
  • Neutrophils / pathology
  • Nose Neoplasms / complications*
  • Nose Neoplasms / diagnosis
  • Nose Neoplasms / immunology
  • Nose Neoplasms / pathology

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Myeloblastin