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Abstract: Epstein-Barr virus-positive (EBV+) post-transplant lymphoproliferative disease (PTLD) is an ultra-rare and aggressive condition that may occur following allogeneic hematopoietic cell transplant (HCT) due to immunosuppression. Approximately half of EBV+ PTLD cases are relapsed or refractory (R/R) to initial rituximab-containing therapy. There are limited treatment options and no standard of care for patients with R/R EBV+ PTLD, and little is known about their treatment history and outcomes. We performed a multinational, multicenter, retrospective chart review of patients with R/R EBV+ PTLD following HCT to describe patients' demographic and disease characteristics, treatment history, and overall survival (OS) from rituximab failure. Among 81 patients who received initial treatment with rituximab as monotherapy (84.0%) or in combination with chemotherapy (16.0%), median time from HCT to PTLD diagnosis was 3.0 months and median OS was 0.7 months. Thirty-six patients received a subsequent line of treatment. The most frequent causes of death were PTLD (56.8%), graft-versus-host disease (13.5%) and treatment-related mortality (10.8%). In multivariate analysis, early PTLD onset and lack of response to initial treatment were associated with mortality. This real-world study demonstrates that the prognosis of patients with R/R EBV+ PTLD following HCT remains poor, highlighting the urgent unmet medical need in this population.
Fuente: Bone Marrow Transplantation, 2023
Editorial: Nature Publishing Group
Año de publicación: 2023
Nº de páginas: 7
Tipo de publicación: Artículo de Revista
DOI: 10.1038/s41409-023-02127-9
ISSN: 0268-3369,1476-5365
Url de la publicación: https://doi.org/10.1038/s41409-023-02127-9
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SOCIÉ, GÉRARD
BARBA, PERE
BARLEV, ARIE
SANZ, JAIME
GARCÍA-CADENAS, IRENE
CHEVALLIER, PATRICE
FAGIOLI, FRANCA
GUZMÁN-BECERRA, NORMA
KUMAR , DEEPALI
LJUNGMAN, PER
PIGNEUX, ARNAUD
SADETSKY, NATALIA
LUCRECIA YAÑEZ SAN SEGUNDO
SHADMAN, MAZYAR
STOREK, JAN
THIRUMALAI, DHANALAKSHMI
XING, BAODONG
MOTHY, MOHAMED
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