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Multicenter Study
. 2013 Sep;57(6):794-802.
doi: 10.1093/cid/cit391. Epub 2013 Jun 13.

Response to rituximab-based therapy and risk factor analysis in Epstein Barr Virus-related lymphoproliferative disorder after hematopoietic stem cell transplant in children and adults: a study from the Infectious Diseases Working Party of the European Group for Blood and Marrow Transplantation

Jan Styczynski  1 , Lidia Gil, Gloria Tridello, Per Ljungman, J Peter Donnelly, Walter van der Velden, Hamdy Omar, Rodrigo Martino, Constantijn Halkes, Maura Faraci, Koen Theunissen, Krzysztof Kalwak, Petr Hubacek, Simona Sica, Chiara Nozzoli, Franca Fagioli, Susanne Matthes, Miguel A Diaz, Maddalena Migliavacca, Adriana Balduzzi, Agnieszka Tomaszewska, Rafael de la Camara, Anja van Biezen, Jennifer Hoek, Simona Iacobelli, Hermann Einsele, Simone Cesaro; Infectious Diseases Working Party of the European Group for Blood and Marrow Transplantation
Affiliations
Multicenter Study

Response to rituximab-based therapy and risk factor analysis in Epstein Barr Virus-related lymphoproliferative disorder after hematopoietic stem cell transplant in children and adults: a study from the Infectious Diseases Working Party of the European Group for Blood and Marrow Transplantation

Jan Styczynski et al. Clin Infect Dis. 2013 Sep.

Abstract

Background: The objective of this analysis was to investigate prognostic factors that influence the outcome of Epstein-Barr virus (EBV)-related posttransplant lymphoproliferative disorder (PTLD) after a rituximab-based treatment in the allogeneic hematopoietic stem cell transplant (HSCT) setting.

Methods: A total of 4466 allogeneic HSCTs performed between 1999 and 2011 in 19 European Group for Blood and Marrow Transplantation centers were retrospectively analyzed for PTLD, either biopsy-proven or probable disease.

Results: One hundred forty-four cases of PTLD were identified, indicating an overall EBV-related PTLD frequency of 3.22%, ranging from 1.16% for matched-family donor, 2.86% for mismatched family donor, 3.97% in matched unrelated donors, and 11.24% in mismatched unrelated donor recipients. In total, 69.4% patients survived PTLD. Multivariable analysis showed that a poor response of PTLD to rituximab was associated with an age ≥30 years, involvement of extralymphoid tissue, acute GVHD, and a lack of reduction of immunosuppression upon PTLD diagnosis. In the prognostic model, the PTLD mortality increased with the increasing number of factors: 0-1, 2, or 3 factors being associated with mortality of 7%, 37%, and 72%, respectively (P < .0001). Immunosuppression tapering was associated with a lower PTLD mortality (16% vs 39%), and a decrease of EBV DNAemia in peripheral blood during therapy was predictive of better survival.

Conclusions: More than two-thirds of patients with EBV-related PTLD survived after rituximab-based treatment. Reduction of immunosuppression was associated with improved outcome, whereas older age, extranodal disease, and acute graft-vs-host disease predicted poor outcome.

Keywords: Epstein-Barr virus; hematopoietic stem cell transplantation; post-transplant lymphoproliferative disorder; prognostic model; risk factors.

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