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A successful treatment for chronic active Epstein-Barr virus disease with Nephrotic Syndrome

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Abstract

Chronic active Epstein-Barr virus (CAEBV) disease is more likely to occur when a patient is on immunosuppressive therapy for any disease or is susceptible to infection, and the prognosis is poor without appropriate treatment, including hematopoietic stem cell transplantation (HSCT). In addition to HSCT, several other chemotherapy regimens have been reported, but all of them are difficult to maintain in remission. Without HSCT, survival rates have been reported to be 50% in 5 years and 25% in 15 years. This is a report of a 13-year-old boy who developed CAEBV disease during cyclosporine A (CyA) treatment for the steroid-dependent nephrotic syndrome (SDNS). Since SDNS precluded HSCT or chemotherapy, CyA was tapered off based on the belief that alleviating his immunosuppressed state would decrease the CAEBV disease. We decided to gradually reduce the CyA dose to activate T-cell immunity, while periodically monitoring the EBV viral load. Finally, we found an appropriate dose that could suppress both CAEBV disease and SDNS, and it lasted for more than 9 years. No case has been reported to date in which a patient developed CAEBV disease while receiving immunosuppressive drugs for the primary disease, and both diseases were controlled only by reducing the dose of immunosuppressive drugs. In this report, we show that dose reduction of immunosuppressive agents without chemotherapy or HSCT is an effective option for the treatment of CAEBV disease in patients receiving immunosuppressive agents.

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Acknowledgements

This work was funded by the Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences.

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Authors and Affiliations

  1. Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, 8-35-1 Sakuragaoka, Kagoshima City, 890-8520, Japan

    Yasuhiro Inaba, Akinori Miyazono & Yasuhiro Okamoto

  2. Department of Advanced Medicine for Infections, National Center for Child Health and Development (NCCHD), Tokyo, Japan

    Kenichi Imadome

  3. Department of Pediatrics, National Hospital Organization Ibuski Medical Center, Ibuski, Japan

    Shinji Aratake

Authors
  1. Yasuhiro Inaba
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  2. Akinori Miyazono
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  3. Kenichi Imadome
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  4. Shinji Aratake
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  5. Yasuhiro Okamoto
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Contributions

The original draft was written by YI, and all authors reviewed the draft. The final manuscript was read and approved by all authors. Viral quantitative testing for EBV was performed by KI.

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Correspondence to Yasuhiro Inaba.

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All the authors have declared no competing interest.

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This article does not contain any studies with human participants or animals performed by any of the authors.

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Inaba, Y., Miyazono, A., Imadome, K. et al. A successful treatment for chronic active Epstein-Barr virus disease with Nephrotic Syndrome. CEN Case Rep (2023). https://doi.org/10.1007/s13730-023-00815-5

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  • DOI: https://doi.org/10.1007/s13730-023-00815-5

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