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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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.
Conflict of interest
The authors declare no conflict of interest.
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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
- Chronic active Epstein–Barr virus infection
- Hypersensitivity to mosquito bites
- Hydroa vacciniforme
- EBV-associated T/NK-cell lymphoproliferative diseases
- Allogeneic hematopoietic stem cell transplantation