Peripheral T cell lymphoma with a high titer of proteinase-3-antineutrophil cytoplasmic antibodies that resembled Wegener's granulomatosis.
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.
Shirai
Tsuyoshi
T
Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai.
Takahashi
Reiko
R
Tajima
Yumi
Y
Kohata
Katsura
K
Yamamoto
Joji
J
Fujii
Hiroshi
H
Takasawa
Naruhiko
N
Ishizawa
Kenichi
K
Ichinohasama
Ryo
R
Ishii
Tomonori
T
Harigae
Hideo
H
D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D003937:Diagnosis, Differential; D014890:Granulomatosis with Polyangiitis / Q000097:blood* / Q000175:diagnosis*; D006801:Humans; D016411:Lymphoma, T-Cell, Peripheral / Q000097:blood* / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D053146:Myeloblastin / Q000097:blood*
D019268:Antibodies, Antineutrophil Cytoplasmic; D053146:Myeloblastin