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. Am. J. Hematol. 83:721-727, 2008. V

EBV-associated T/NK–cell lymphoproliferative diseases in nonimmunocompromised hosts: prospective analysis of 108 cases

“…Patients with CAEBV have a higher risk of transplantation-related complications. 41,48 Recently, Kawa et al reported excellent outcome of HSCT with reduced-intensity conditioning. 40 Although the superiority of reduced-intensity conditioning over myeloablative conditioning did not reach statistical significance in that study, it appears that a reduced-intensity regimen is sufficient to prevent transplantation-related deaths.…”

Characterization and treatment of chronic active Epstein-Barr virus disease: a 28-year experience in the United States

“…In contrast, allogeneic HSCT has been successful in several cases reported from Japan. [12][13][14] We report our experience with 19 patients with CAEBV. Sixteen consecutive patients were followed at the National Institutes of Health (NIH) Clinical Center during the past 28 years, and 3 patients were seen at Baylor College of Medicine.…”

Quantification of Epstein-Barr Virus DNA Is Helpful for Evaluation of Chronic Active Epstein-Barr Virus Infection

“…Most patients are treated with antiviral agents (acyclovir and ganciclovir), cytokines (Interferon-α and Interleukin-2), and chemotherapy (corticosteroids, etoposide, and cyclosporine). Recently, promising results have been obtained with autologous EBV-specific cytotoxic T cell therapy and hematopoietic cell transplantation (Savoldo et al 2002;Gotoh et al 2008;Sato et al 2008). The chemotherapy reduced the EBV DNA levels of this patient.…”