How we treat chronic active Epstein–Barr virus infection

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 c...

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Bibliographic Details
Published in:International Journal of Hematology 2017-04, Vol.105 (4), p.406-418
Main Authors: Sawada, Akihisa, Inoue, Masami, Kawa, Keisei
Format: Article
Language:English
Subjects:
Adult
Allografts
Child
Chronic Disease
Epstein-Barr virus
Epstein-Barr Virus Infections - mortality
Epstein-Barr Virus Infections - therapy
Female
Hematology
Hematopoietic Stem Cell Transplantation
Humans
Lymphoproliferative Disorders - mortality
Lymphoproliferative Disorders - therapy
Lymphoproliferative Disorders - virology
Male
Medicine
Medicine & Public Health
Oncology
Review Article
Survival Rate
Young Adult
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Summary: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.
ISSN:0925-5710
1865-3774
DOI:10.1007/s12185-017-2192-6