Summary:
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/m2), and cyclophosphamide (120 mg/kg) or melphalan (210 mg/m2). 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.
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Acknowledgements
We thank Dr Cliona M Rooney and Helen E Heslop, Center for Cell and Gene Therapy, Baylor College of Medicine, for critical reading of the manuscript.
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Okamura, T., Kishimoto, T., Inoue, M. et al. Unrelated bone marrow transplantation for Epstein–Barr virus-associated T/NK-cell lymphoproliferative disease. Bone Marrow Transplant 31, 105–111 (2003). https://doi.org/10.1038/sj.bmt.1703796
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DOI: https://doi.org/10.1038/sj.bmt.1703796
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