A Case of NK/T Cell Lymphoma Mimicking Limited Form of Wegener's Granulomatosis.
10.4078/jkra.2010.17.3.289
- Author:
Yun Jung PARK
1
;
Chul Su CHO
Author Information
1. Division of Rheumatology, Department of Internal Medicine, The Catholic University of Korea, Seoul, Korea. chocs@catholic.ac.kr
- Publication Type:Case Report
- Keywords:
Limited form of Wegenr's granulomatosis;
NK/T cell lymphoma
- MeSH:
Biopsy;
Cyclophosphamide;
Foot;
Humans;
Lymphoma;
Masks;
Necrosis;
Organic Chemicals;
Peripheral Nerves;
Prognosis;
Recurrence;
Sural Nerve;
Tuberculosis;
Vasculitis;
Wegener Granulomatosis
- From:The Journal of the Korean Rheumatism Association
2010;17(3):289-294
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
It is occasionally difficult to distinguish Wegener's granulomatosis (WG) from other diseases including malignancy, tuberculosis, and various types of vasculitis because of the overlapping symptoms and signs. We report on a patient with NK/T cell lymphoma who was treated with a limited form of WG. At his first visit, he presented with left foot drop and recurrent nasal swelling. Necrosis and massive infiltration of inflammatory cells were identified on a nasal tissue biopsy. Sural nerve biopsy findings also showed infiltration of inflammatory cells in both the endoneurium and perivascular area; thus, a diagnosis of a limited form of WG was made. After combination therapy with a glucocorticoid and oral cyclophosphamide was initiated, his condition completely recovered without recurrence for the next 2 years. However, he visited the hospital again for recurrence of nasal swelling. Repeated biopsy of nasal tissues, combined with an immunophenotypic analysis revealed NK/T cell lymphoma. The possibility of NK/T lymphoma should be considered when evaluating a limited type of WG, which shows atypical findings on biopsy as well as recurrent deterioration, as a suboptimal dose of immunosuppressive therapy may mask its expression and lead to a poor prognosis.
