Peripheral T cell lymphoma with a high titer of proteinase-3-antineutrophil cytoplasmic antibodies that resembled Wegener's granulomatosis.

Abstract

Here, we present a 54-year-old man with proptosis and swelling below the left eyelid. Laboratory findings showed high levels of PR3-ANCA and histological examination of the first biopsy revealed acute inflammation. Together with the findings of MRI, a diagnosis of WG was made. However, the disease progressed rapidly and histological examination of the second biopsy revealed infiltration of neoplastic T lymphocytes with aberrant loss of CD7. A final diagnosis of peripheral T cell lymphoma, not otherwise specified (WHO) was made, and complete remission was achieved by chemotherapy. This is a very rare case of T cell lymphoma with a high titer of PR3-ANCA.

• Authors: Tsuyoshi Shirai, Reiko Takahashi, Yumi Tajima, Katsura Kohata, Joji Yamamoto, Hiroshi Fujii, Naruhiko Takasawa, Kenichi Ishizawa, Ryo Ichinohasama, Tomonori Ishii, Hideo Harigae
• Journal: Internal medicine (Tokyo, Japan) (Intern Med) Vol. 48 Issue 23 Pg. 2041-5 ( 2009) ISSN: 1349-7235 [Electronic] Japan
• PMID: 19952489 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antibodies, Antineutrophil Cytoplasmic
• Myeloblastin

Topics

• Antibodies, Antineutrophil Cytoplasmic (blood)
• Diagnosis, Differential
• Granulomatosis with Polyangiitis (blood, diagnosis)
• Humans
• Lymphoma, T-Cell, Peripheral (blood, diagnosis)
• Male
• Middle Aged
• Myeloblastin (blood)