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How we treat chronic active Epstein–Barr virus infection

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Abstract

Chronic active Epstein–Barr virus infection (CAEBV) is a prototype of the EBV-associated T- or NK-cell lymphoproliferative diseases, which also include hypersensitivity to mosquito bites and severe-type hydroavacciniforme. The manifestations of CAEBV are often self-limiting with minimum supportive care or only prednisolone and cyclosporine A with or without etoposide. However, allogeneic hematopoietic stem cell transplantation (HSCT) is the only cure, without which patients with CAEBV die within several years. A severe hypercytokinemia and hemophagocytic syndrome, which may occur suddenly, often results in a fatal clinical course. At out institute, we have established a 3-step strategy, including allogeneic HSCT, for the treatment of CAEBV. Seventy-nine patients with CAEBV and related diseases have been treated to date. The 3-year overall survival rate (3y-OS) is currently 87.3 ± 4.2% after planned allogeneic HSCT. However, 3y-OS in patients with uncontrolled active disease is only 16.7 ± 10.8%. To maximize survival rates with minimized late sequelae, we recommend earlier initiation and completion of the 3-step treatment without watchful waiting. We present six illustrative and difficult cases (including severe hypercytokinemia or emergent HSCT) and discuss them together with 73 residual cases.

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Acknowledgements

The authors thank Dr. Takayuki Okamura and Dr. Emiko Sato-Miyashita for their contributions to early studies. The authors thank Dr. Maho Koyama-Sato for establishing the ESCAP regimen. The authors thank Prof. Hiroshi Kimura and Prof. Ken-ichiImadome for their pathological cooperation. The authors thank all patients and staff included in this review.

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Authors and Affiliations

  1. Department of Hematology/Oncology, Osaka Medical Center and Research Institute for Maternal and Child Health, 840 Murodo, Izumi City, Osaka, 594-1101, Japan

    Akihisa Sawada, Masami Inoue & Keisei Kawa

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  1. Akihisa Sawada
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  2. Masami Inoue
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  3. Keisei Kawa
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Correspondence to Akihisa Sawada.

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Sawada, A., Inoue, M. & Kawa, K. How we treat chronic active Epstein–Barr virus infection. Int J Hematol 105, 406–418 (2017). https://doi.org/10.1007/s12185-017-2192-6

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  • DOI: https://doi.org/10.1007/s12185-017-2192-6

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