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Haematologica. 2016 Jul;101(7):803-11. doi: 10.3324/haematol.2016.144428.

Management of Epstein-Barr Virus infections and post-transplant lymphoproliferative disorders in patients after allogeneic hematopoietic stem cell transplantation: Sixth European Conference on Infections in Leukemia (ECIL-6) guidelines.

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Department of Pediatric Hematology and Oncology, Collegium Medicum, Nicolaus Copernicus University Torun, Jurasz University Hospital, Bydgoszcz, Poland
Department of Hematology, Radboud University Medical Centre, Nijmegen, The Netherlands.
Center for Clinical Hematology, Nottingham University Hospitals NHS Trust, Nottingham, UK.
Department of Pediatrics, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
Hospital de la Princesa, Department of Hematology, Madrid, Spain.
Department of Hematology, Henri Mondor Hospital, Assistance Publique-Hôpitaux de Paris, and University Paris-Est-Créteil, Créteil, France.
Karolinska University Hospital, Departments of Hematology and Allogeneic Stem Cell Transplantation, Karolinska Institutet, Division of Hematology, Department of Medicine, Huddinge, Stockholm, Sweden.


Epstein-Barr virus-related post-transplant lymphoproliferative disorders are recognized as a significant cause of morbidity and mortality in patients undergoing hematopoietic stem cell transplantation. To better define current understanding of post-transplant lymphoproliferative disorders in stem cell transplant patients, and to improve its diagnosis and management, a working group of the Sixth European Conference on Infections in Leukemia 2015 reviewed the literature, graded the available quality of evidence, and developed evidence-based recommendations for diagnosis, prevention, prophylaxis and therapy of post-transplant lymphoproliferative disorders exclusively in the stem cell transplant setting. The key elements in diagnosis include non-invasive and invasive methods. The former are based on quantitative viral load measurement and imaging with positron emission tomography; the latter with tissue biopsy for histopathology and detection of Epstein-Barr virus. The diagnosis of post-transplant lymphoproliferative disorder can be established on a proven or probable level. Therapeutic strategies include prophylaxis, preemptive therapy and targeted therapy. Rituximab, reduction of immunosuppression and Epstein-Barr virus-specific cytotoxic T-cell therapy are recommended as first-line therapy, whilst unselected donor lymphocyte infusions or chemotherapy are options as second-line therapy; other methods including antiviral drugs are discouraged.

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