title: Allogeneic hematopoietic stem cell transplantation for Epstein-Barr virus-associated T/natural killer-cell lymphoproliferative disease in Japan.
abstract: Epstein-Barr virus (EBV)-associated T/NK-cell lymphoproliferative disease (LPD) has been linked to several different disorders. Its prognosis is generally poor and a treatment strategy has yet to be established. There are reports, however, that hematopoietic stem cell transplantation (HSCT) can cure this disease. To clarify the current situation regarding allogeneic hematopoietic stem cell transplantation (allo-HSCT) for EBV-associated T/NK-LPD, a nationwide survey was performed in Japan. Data for 74 patients were collected. There were 42 cases of chronic active EBV infection (CAEBV), 10 cases of EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH), and 22 cases of EBV-associated lymphoma/leukemia (EBV-lymphoma/leukemia). Of those with CAEBV, 54% had the EBV-infected T-cell type and 59% with EBV-lymphoma/leukemia had the EBV-infected NK-cell type. Most patients with EBV-HLH and EBV-lymphoma/leukemia received allo-HSCT within 1 year after onset compared to only 14% of patients with CAEBV. The event-free survival (EFS) rate following allo-HSCT was 0.561 +/- 0.086 for CAEBV, 0.614 +/- 0.186 for EBV-HLH, and 0.309 +/- 0.107 for EBV-lymphoma/leukemia. The EFS of allo-HSCT with conventional conditioning was 0.488 +/- 0.074 and with reduced-intensity conditioning was 0.563 +/- 0.124. Thus, in a substantial number of cases, EBV-associated T/NK-LPD can be cured by either allogeneic conventional stem cell transplantation or reduced-intensity stem cell transplantation.
authors.affiliation: Department of Pediatrics, Osaka Medical Center and Research Institute for Maternal and Child Health, 840 Murodo-cho, Izumi, Osaka, Japan. satoemik@s3.dion.ne.jp
authors.forename: Emiko
authors.initials: E
authors.lastname: Sato
authors.forename: Shouichi
authors.initials: S
authors.lastname: Ohga
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kuroda
authors.forename: Fumiaki
authors.initials: F
authors.lastname: Yoshiba
authors.forename: Miki
authors.initials: M
authors.lastname: Nishimura
authors.forename: Masayuki
authors.initials: M
authors.lastname: Nagasawa
authors.forename: Masami
authors.initials: M
authors.lastname: Inoue
authors.forename: Keisei
authors.initials: K
authors.lastname: Kawa
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007223:Infant; D007564:Japan / Q000453:epidemiology; D007694:Killer Cells, Natural / Q000473:pathology; D054066:Leukemia, Large Granular Lymphocytic / Q000453:epidemiology / Q000473:pathology / Q000601:surgery / Q000821:virology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology; D008228:Lymphoma, Non-Hodgkin / Q000453:epidemiology / Q000473:pathology / Q000601:surgery / Q000821:virology; D008232:Lymphoproliferative Disorders / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology / Q000601:surgery* / Q000821:virology; D008297:Male; D008875:Middle Aged; D013601:T-Lymphocytes / Q000473:pathology; D019172:Transplantation Conditioning; D014184:Transplantation, Homologous; D016896:Treatment Outcome