title: How we treat chronic active Epstein-Barr virus infection.
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.
authors.affiliation: Department of Hematology/Oncology, Osaka Medical Center and Research Institute for Maternal and Child Health, 840 Murodo, Izumi City, Osaka, 594-1101, Japan. asawada@mch.pref.osaka.jp.
authors.forename: Akihisa
authors.initials: A
authors.lastname: Sawada
authors.affiliation: Department of Hematology/Oncology, Osaka Medical Center and Research Institute for Maternal and Child Health, 840 Murodo, Izumi City, Osaka, 594-1101, Japan.
authors.forename: Masami
authors.initials: M
authors.lastname: Inoue
authors.affiliation: Department of Hematology/Oncology, Osaka Medical Center and Research Institute for Maternal and Child Health, 840 Murodo, Izumi City, Osaka, 594-1101, Japan.
authors.forename: Keisei
authors.initials: K
authors.lastname: Kawa
mesh_terms: D000328:Adult; D064591:Allografts; D002648:Child; D002908:Chronic Disease; D020031:Epstein-Barr Virus Infections / Q000401:mortality / Q000628:therapy*; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000401:mortality / Q000628:therapy* / Q000821:virology; D008297:Male; D015996:Survival Rate; D055815:Young Adult
keywords: Allogeneic hematopoietic stem cell transplantation; Chronic active Epstein–Barr virus infection; EBV-associated T/NK-cell lymphoproliferative diseases; Hydroa vacciniforme; Hypersensitivity to mosquito bites