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Management of PTLD After HSCT

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Post-Transplant Lymphoproliferative Disorders

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Abstract

Post-transplant lymphoproliferative disorders (PTLDs) are life-threatening complications after hematopoietic stem cell transplantation (HSCT), often associated to Epstein-Barr virus (EBV) infection. Rituximab, reduction of immunosuppression, and EBV-specific cytotoxic T-lymphocyte (CTL) therapy are recommended as first-line therapy, while unselected donor lymphocyte infusions (DLI) and chemotherapy are options as second-line therapy. Other agents, including antiviral drugs, are discouraged. Pooling results from published studies in HSCT recipients reveals that administration of rituximab results in a positive outcome in more than 65% of patients treated for EBV-associated PTLD. RIS, when applied in combination with rituximab, improved the results to a rate of 80%. The use of EBV-CTLs was successful in approximately 75% of subjects with EBV-associated PTLD. EBV-negative B- and T-cell PTLD cases develo** late after HSCT (>5 years off immunosuppression) should be regarded as malignant lymphomas and should, therefore, be treated using protocols for de novo lymphoma occurring in the immunocompetent host. Novel therapeutic options are being tested in clinical trials and may contribute to ameliorate outcome in PTLD entities with poor prognosis.

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Authors and Affiliations

  1. Pediatric Hematology/Oncology and Cell Factory, IRCCS Fondazione Policlinico San Matteo, Pavia, Italy

    Patrizia Comoli

  2. Department of Pediatric Hematology and Oncology, Collegium Medicum, Nicolaus Copernicus University Torun, Jurasz University Hospital, Bydgoszcz, Poland

    Jan Styczynski

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  1. Patrizia Comoli
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  2. Jan Styczynski
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Correspondence to Patrizia Comoli .

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Editors and Affiliations

  1. Washington University School of Medicine and St. Louis Children’s Hospital, Saint Louis, MO, USA

    Vikas R. Dharnidharka

  2. UPMC Children’s Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA, USA

    Michael Green

  3. Vanderbilt University Medical Center, Nashville, TN, USA

    Steven A. Webber

  4. Ev. Diakonie-Krankenhaus Bremen, German PTLD Study Group, Bremen, Bremen, Germany

    Ralf Ulrich Trappe

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Comoli, P., Styczynski, J. (2021). Management of PTLD After HSCT. In: Dharnidharka, V.R., Green, M., Webber, S.A., Trappe, R.U. (eds) Post-Transplant Lymphoproliferative Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-65403-0_14

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