title: Unrelated bone marrow transplantation for Epstein-Barr virus-associated T/NK-cell lymphoproliferative disease.
abstract: Epstein-Barr virus (EBV)-associated T/NK-cell lymphoproliferative disease (LPD) has been linked to several different disorders, including chronic active EBV infection, EBV-associated hemophagocytic syndrome, hypersensitivity to mosquito bites, hydroa vacciniforme, aggressive NK-cell leukemia, and nasal/nasal-type NK-cell lymphoma. In most instances, these disorders are refractory to conventional treatments and have a poor prognosis. Here, we report a new treatment strategy for EBV-associated T/NK-cell LPD, consisting of immunochemotherapy, intensive combination chemotherapy, and stem cell transplantation. The five patients studied, two with T-cell and three with NK-cell LPD, lacked a human leukocyte antigen-matched, related donor, and therefore received bone marrow grafts from HLA-matched, unrelated donors. The preconditioning regimen consisted of total-body irradiation (12 Gy), etoposide (900 mg/m(2)), and cyclophosphamide (120 mg/kg) or melphalan (210 mg/m(2)). All patients had residual LPD by a quantitative PCR technique prior to transplantation. After unrelated bone marrow transplantation (UBMT), four of the five patients remain in continuous complete remission at a median of 19 months, without detectable EBV-DNA in peripheral blood. Thus, UBMT appears to be a reasonable option for the treatment of patients with EBV-associated T/NK-cell LPD. Detection of EBV-DNA by PCR offers an important tool for assessing minimal residual disease in patients with EBV-associated T/NK-cell LPD.
authors.affiliation: Department of Pediatrics, Osaka Medical Center, Osaka, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Okamura
authors.forename: T
authors.initials: T
authors.lastname: Kishimoto
authors.forename: M
authors.initials: M
authors.lastname: Inoue
authors.forename: M
authors.initials: M
authors.lastname: Honda
authors.forename: N
authors.initials: N
authors.lastname: Yamashita
authors.forename: H
authors.initials: H
authors.lastname: Wakiguchi
authors.forename: M
authors.initials: M
authors.lastname: Yagita
authors.forename: G
authors.initials: G
authors.lastname: Hosoi
authors.forename: M
authors.initials: M
authors.lastname: Sako
authors.forename: M
authors.initials: M
authors.lastname: Yasui
authors.forename: K
authors.initials: K
authors.lastname: Yagi
authors.forename: K
authors.initials: K
authors.lastname: Kawa
mesh_terms: D000293:Adolescent; D016026:Bone Marrow Transplantation / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D004279:DNA, Viral / Q000235:genetics / Q000302:isolation & purification; D004359:Drug Therapy, Combination; D005260:Female; D004854:Herpesvirus 4, Human / Q000302:isolation & purification* / Q000472:pathogenicity; D006801:Humans; D007694:Killer Cells, Natural / Q000276:immunology* / Q000821:virology; D008232:Lymphoproliferative Disorders / Q000628:therapy* / Q000821:virology; D008297:Male; D016133:Polymerase Chain Reaction; D013601:T-Lymphocytes / Q000276:immunology* / Q000821:virology; D019172:Transplantation Conditioning / Q000379:methods; D014184:Transplantation, Homologous; D016896:Treatment Outcome
chemical_list: D004279:DNA, Viral