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A case of lymphomatoid granulomatosis with central nervous system involvement successfully treated with IFNα

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Abstract

Lymphomatoid granulomatosis (LYG) is a rare Epstein–Barr virus (EBV)-driven B-cell lymphoproliferative disease affecting mainly extranodal sites such as the lung, central nervous system (CNS), skin, kidney, and liver. We report a case of low-grade LYG involving the CNS that was successfully treated with interferon alpha (IFNα). A 69-year-old woman developed necrotic erythema of the skin and was initially diagnosed with pyoderma gangrenosum based on skin biopsy. She showed a limited response to prednisolone. Approximately 6 months after the initial onset, low-grade LYG was diagnosed after detection of CNS lesions on brain biopsy. The whole blood EBV-DNA load determined by real-time polymerase chain reaction was slightly elevated. Two months into IFNα therapy, skin and CNS lesions had responded favorably and the EBV-DNA load decreased. IFNα plays an important role in treatment of LYG through its antiproliferative, immunomodulatory, and anti-EBV effects. To our knowledge, this is the first case report of successful treatment with IFNα in Japan. Further investigation is necessary to determine optimal use of IFNα for LYG.

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Acknowledgements

We would like to express our gratitude to Fumiko Arakawa, Ph.D. of Department of Pathology, Kurume University School of Medicine, for technical support with the PCR experiments.

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Authors and Affiliations

  1. Division of Hematology and Oncology, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan

    Yoshitaka Yamasaki, Satoshi Morishige, Shuki Oya, Takayuki Nakamura, Maki Yamaguchi, Kazutoshi Aoyama, Fumihiko Mouri, Koichi Osaki & Koji Nagafuji

  2. Department of Neurosurgery, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan

    Satoru Komaki & Motohiro Morioka

  3. Department of Pathology, School of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan

    Takuya Furuta & Koichi Ohshima

  4. Department of Dermatology, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan

    Hiroshi Koga & Takekuni Nakama

Authors
  1. Yoshitaka Yamasaki
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  2. Satoshi Morishige
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  3. Satoru Komaki
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  7. Takayuki Nakamura
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  9. Kazutoshi Aoyama
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  10. Fumihiko Mouri
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  13. Koichi Ohshima
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  14. Motohiro Morioka
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  15. Koji Nagafuji
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Correspondence to Satoshi Morishige.

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Yamasaki, Y., Morishige, S., Komaki, S. et al. A case of lymphomatoid granulomatosis with central nervous system involvement successfully treated with IFNα. Int J Hematol 114, 502–508 (2021). https://doi.org/10.1007/s12185-021-03178-8

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  • DOI: https://doi.org/10.1007/s12185-021-03178-8

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