A 79-year-old man presented to the dental department with a 1-week history of a necrotic
ulcer on the midline of the gingiva (Figure 1A). A biopsy specimen resulted in a diagnosis of gingivitis and he was prescribed with
antibiotics, which did not heal the ulcer. Three weeks later, he was referred to the
otorhinolaryngology department with odynophagia caused by the ulcer in the midline
of the soft palate (Figure 1B). Endoscopic examination of the upper respiratory tract also found eroded nasal mucosa
(Figure 1C). Laboratory tests revealed slightly elevated Epstein-Barr virus (EBV)-DNA load of
80 copy/106 cells (normal value, <20) although proteinase 3 and myeloperoxidase antineutrophil
cytoplasmic antibodies were negative. Further biopsies of the gingiva, soft palate,
and nasal cavity showed infiltrative CD56 and EBV-encoded small RNA-positive atypical
lymphocytes. Extranodal natural killer/T-cell lymphoma, nasal type (ENKL), which was
once described as “lethal midline granuloma,”
1
was diagnosed. He underwent chemoradiotherapy and achieved complete remission. However,
the femoral skin swelled 2 years later and blood tests revealed re-elevated EBV-DNA
load of 34,000 copy/106 cells. A biopsy confirmed a diagnosis of relapsed lymphoma. He selected best supportive
care and died 2 months later.
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to The American Journal of the Medical SciencesAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
REFERENCES
- Epstein-Barr virus in nasal T-cell lymphomas in patients with lethal midline granuloma.Lancet. 1990; 335: 128-130
- Extranodal NK/T-cell lymphoma: diagnosis and treatment cues.Hematol Oncol. 2008; 26: 66-72
- Extranodal natural killer/T-cell lymphoma, nasal type: basic science and clinical progress.Front Pediatr. 2019; 7: 141
Article info
Publication history
Published online: March 18, 2020
Accepted:
March 11,
2020
Received:
January 30,
2020
Footnotes
The authors have no financial or other conflicts of interest to disclose.
Identification
Copyright
© 2020 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.
