Allogeneic hematopoietic stem cell transplantation for Epstein–Barr virus-associated T/natural killer-cell lymphoproliferative disease in Japan
Article first published online: 18 JUN 2008
Copyright © 2008 Wiley-Liss, Inc.
American Journal of Hematology
Volume 83, Issue 9, pages 721–727, September 2008
How to Cite
Sato, E., Ohga, S., Kuroda, H., Yoshiba, F., Nishimura, M., Nagasawa, M., Inoue, M. and Kawa, K. (2008), Allogeneic hematopoietic stem cell transplantation for Epstein–Barr virus-associated T/natural killer-cell lymphoproliferative disease in Japan. Am. J. Hematol., 83: 721–727. doi: 10.1002/ajh.21247
- Issue published online: 24 AUG 2008
- Article first published online: 18 JUN 2008
- Accepted manuscript online: 18 JUN 2008 12:00AM EST
- Manuscript Accepted: 12 JUN 2008
- Manuscript Revised: 4 MAY 2008
- Manuscript Received: 7 FEB 2008
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. © 2008 Wiley-Liss, Inc. Am. J. Hematol., 2008.