title: 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.
abstract: 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.
authors.affiliation: Department of Pediatric Hematology and Oncology, Collegium Medicum, Nicolaus Copernicus University Torun, Jurasz University Hospital, Bydgoszcz, Poland jstyczynski@cm.umk.pl.
authors.forename: Jan
authors.initials: J
authors.lastname: Styczynski
authors.affiliation: Department of Hematology, Radboud University Medical Centre, Nijmegen, The Netherlands.
authors.forename: Walter
authors.initials: W
authors.lastname: van der Velden
authors.affiliation: Center for Clinical Hematology, Nottingham University Hospitals NHS Trust, Nottingham, UK.
authors.forename: Christopher P
authors.initials: CP
authors.lastname: Fox
authors.affiliation: Department of Pediatrics, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
authors.forename: Dan
authors.initials: D
authors.lastname: Engelhard
authors.affiliation: Hospital de la Princesa, Department of Hematology, Madrid, Spain.
authors.forename: Rafael
authors.initials: R
authors.lastname: de la Camara
authors.affiliation: Department of Hematology, Henri Mondor Hospital, Assistance Publique-Hôpitaux de Paris, and University Paris-Est-Créteil, Créteil, France.
authors.forename: Catherine
authors.initials: C
authors.lastname: Cordonnier
authors.affiliation: Karolinska University Hospital, Departments of Hematology and Allogeneic Stem Cell Transplantation, Karolinska Institutet, Division of Hematology, Department of Medicine, Huddinge, Stockholm, Sweden.
authors.forename: Per
authors.initials: P
authors.lastname: Ljungman
mesh_terms: D019468:Disease Management; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000453:epidemiology / Q000209:etiology* / Q000517:prevention & control; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy*; D017410:Practice Guidelines as Topic; D012307:Risk Factors