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Chronic active Epstein–Barr virus infection presenting as refractory chronic sinusitis

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Abstract

A 44-year-old Japanese man presented with fever and sore throat. He had a history of refractory chronic sinusitis that did not respond to several years of pharmacotherapy, and underwent endoscopic sinus surgery (ESS) 5 months prior to his presentation, but his symptoms persisted. A biopsy specimen was taken from the right nasal cavity, and extranodal natural killer/T-cell lymphoma, nasal type (ENKTL) was diagnosed. Two years after complete remission was achieved by chemoradiation therapy, he developed hemophagocytic lymphohistiocytosis (HLH) without recurrence of ENKTL. Epstein–Barr virus (EBV)-DNA copy number was relatively high and EBV-infected lymphocytes (CD8 + T cells) were detected in the peripheral blood. Pathological review of the biopsy specimens taken during ESS showed that CD8 + T cells with slightly atypia infiltrating the stroma were EBV positive. These findings suggested that the patient had underlying chronic active EBV infection (CAEBV) that caused the refractory chronic sinusitis, eventually developed into ENKTL, and also caused HLH. Clinicians should consider adult-onset CAEBV in the differential diagnosis of patients with refractory chronic sinusitis.

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Acknowledgements

The manuscript was edited and proofread by Editage (www.editage.jp).

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Authors and Affiliations

  1. Department of Hematology and Oncology, Okayama University Hospital, 2-5-1, Shikata-cho, Kita-ku, Okayama, 700-8558, Japan

    Wataru Kitamura, Hideaki Fujiwara, Akifumi Matsumura, Noboru Asada, Hisakazu Nishimori, Ken-ichi Matsuoka & Yoshinobu Maeda

  2. Department of Otorhinolaryngology, Okayama University Hospital, 2-5-1, Shikata-cho, Kita-ku, Okayama, 700-8558, Japan

    Takaya Higaki

  3. Department of Pathology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, 2-5-1, Shikata-cho, Kita-ku, Okayama, 700-8558, Japan

    Rei Shibata & Tadashi Yoshino

  4. Department of Diagnostic Pathology, Okayama University Hospital, 2-5-1, Shikata-cho, Kita-ku, Okayama, 700-8558, Japan

    Tomohiro Toji

  5. Department of Hematology, Japanese Red Cross Okayama Hospital, 2-1-1, Aoe, Kita-ku, Okayama, 700-8607, Japan

    Soichiro Fujii

  6. Center for Comprehensive Genomic Medicine, Okayama University Hospital, 2-5-1, Shikata-cho, Kita-ku, Okayama, 700-8558, Japan

    Daisuke Ennishi

  7. Division of Clinical Laboratory, Okayama University Hospital, 2-5-1, Shikata-cho, Kita-ku, Okayama, 700-8558, Japan

    Keiko Fujii

  8. Division of Blood Transfusion, Okayama University Hospital, 2-5-1, Shikata-cho, Kita-ku, Okayama, 700-8558, Japan

    Nobuharu Fujii

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  1. Wataru Kitamura
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  2. Hideaki Fujiwara
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  3. Akifumi Matsumura
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  4. Takaya Higaki
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  8. Noboru Asada
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  11. Keiko Fujii
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  12. Nobuharu Fujii
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  14. Tadashi Yoshino
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  15. Yoshinobu Maeda
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Correspondence to Hideaki Fujiwara.

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Kitamura, W., Fujiwara, H., Matsumura, A. et al. Chronic active Epstein–Barr virus infection presenting as refractory chronic sinusitis. Int J Hematol 116, 139–145 (2022). https://doi.org/10.1007/s12185-022-03306-y

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  • DOI: https://doi.org/10.1007/s12185-022-03306-y

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